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1. Reddick WE, Glass JO, Palmer SL, Wu S, Gajjar A, Langston JW, Kun LE, Xiong X, Mulhern RK: Atypical white matter volume development in children following craniospinal irradiation. Neuro Oncol; 2005 Jan;7(1):12-9
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  • Most children with medulloblastoma (MB), the second most common pediatric brain tumor, have a 70% probability of survival.
  • We hypothesized that cranial irradiation inhibited normal brain volume development in these survivors.
  • A quadratic random coefficient model was used to identify trends in brain volume with increasing age.
  • Therefore, volumetric monitoring of brain development can be used to guide the care of survivors, assess the toxicity of previous and current clinical trials, and aid in the design of therapies that minimize toxicity.

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  • (PMID = 15701278.001).
  • [ISSN] 1522-8517
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P30 CA021765; United States / NCI NIH HHS / CA / P30CA21765
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC1871625
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2. Matsumoto J, Kochi M, Morioka M, Nakamura H, Makino K, Hamada J, Kuratsu J, Ushio Y: A long-term ventricular drainage for patients with germ cell tumors or medulloblastoma. Surg Neurol; 2006 Jan;65(1):74-80; discussion 80
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  • [Title] A long-term ventricular drainage for patients with germ cell tumors or medulloblastoma.
  • BACKGROUND: Hydrocephalus associated with intracranial germ cell tumors or disseminated medulloblastoma has been treated with ventriculoperitoneal shunt.
  • However, this procedure has a potential risk of intraperitoneal metastasis of these brain tumors.
  • METHODS: From 1979 to 2003, we have treated 96 patients with germ cell tumors and medulloblastoma in our hospital.
  • Of 96 patients, 59 (germ cell tumor, 31; medulloblastoma, 28) had hydrocephalus and 13 needed long-term cerebrospinal fluid drainage to manage the obstructive hydrocephalus due to persistent tumor or communicating hydrocephalus due to dissemination.
  • We performed PLTVD for these cases using a flow-controlled shunt device and percutaneous long-tunneled shunt tube (peritoneal catheter) exiting at the upper abdomen and connecting to a closed drainage system.
  • CONCLUSIONS: Percutaneous long-tunneled ventricular drainage was an effective method to manage long-lasting obstructive or communicating hydrocephalus with germ cell tumors and medulloblastoma.
  • [MeSH-major] Brain Neoplasms / complications. Hydrocephalus / etiology. Hydrocephalus / surgery. Medulloblastoma / complications. Neoplasms, Germ Cell and Embryonal / complications. Ventriculoperitoneal Shunt
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Incidence. Infant. Infection / epidemiology. Male. Postoperative Complications / epidemiology. Retrospective Studies. Time Factors


3. Furneaux CE, Marshall ES, Yeoh K, Monteith SJ, Mews PJ, Sansur CA, Oskouian RJ, Sharples KJ, Baguley BC: Cell cycle times of short-term cultures of brain cancers as predictors of survival. Br J Cancer; 2008 Nov 18;99(10):1678-83
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  • [Title] Cell cycle times of short-term cultures of brain cancers as predictors of survival.
  • Brain tumour material obtained at surgery from 70 patients with glioblastoma, medulloblastoma, astrocytoma, oligodendroglioma and metastatic melanoma was cultured for 7 days on 96-well plates, coated with agarose to prevent proliferation of fibroblasts.
  • For patients with brain cancers of all types, median survival for the < or =10-day and >10-day groups were 5.1 and 12.5 months, respectively (P=0.0009).
  • Lower grade gliomas had longer median culture cycle times (16 days) than those of medulloblastomas (9.9 days), glioblastomas (9.8 days) or melanomas (6.7 days).
  • We conclude that culture cycle times determined using short-term cultures of surgical material from brain tumours correlate with patient survival.
  • [MeSH-major] Brain Neoplasms / physiopathology. Cell Cycle
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. Female. Humans. Kinetics. Male. Middle Aged. Prognosis. Survival Analysis. Time Factors. Tumor Cells, Cultured

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  • (PMID = 18854836.001).
  • [ISSN] 1532-1827
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2584938
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4. Benesch M, Spiegl K, Winter A, Passini A, Lackner H, Moser A, Sovinz P, Schwinger W, Urban C: A scoring system to quantify late effects in children after treatment for medulloblastoma/ependymoma and its correlation with quality of life and neurocognitive functioning. Childs Nerv Syst; 2009 Feb;25(2):173-81
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  • [Title] A scoring system to quantify late effects in children after treatment for medulloblastoma/ependymoma and its correlation with quality of life and neurocognitive functioning.
  • BACKGROUND: The aim of this study was to quantify the severity of late effects by a simple numerical score (late effects severity score, LESS) in patients who received radiochemotherapy for medulloblastoma or ependymoma.
  • Twenty-three patients with medulloblastoma (n = 18) or ependymoma (n = 5) underwent extensive neurocognitive and QoL testing at a median of 56 months (range, 1-174) after the end of treatment.
  • Eight patients with low-grade glioma (LGG) who underwent tumor resection only served as control group.
  • RESULTS: Patients with medulloblastoma/ependymoma had significantly higher LESS and significantly lower Wechsler Adult Intelligence Scale (WAIS)/Wechsler Intelligence Scales for Children (WISC) scores compared to patients with LGG.
  • Comparison of QoL and late effects in patients with medulloblastoma/ependymoma demonstrated a significant negative correlation only for neurological late effects and the KINDL score suggesting that younger patients with more severe late effects reported on a worse QoL.
  • CONCLUSIONS: This LESS seems to be a simple and practical tool to quantify late effects in former brain tumor patients.
  • [MeSH-major] Ependymoma / therapy. Medulloblastoma / therapy. Nervous System Diseases / physiopathology. Quality of Life
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Cognition / drug effects. Cognition / physiology. Cognition / radiation effects. Combined Modality Therapy / adverse effects. Drug-Related Side Effects and Adverse Reactions. Endocrine System / drug effects. Endocrine System / physiopathology. Endocrine System / radiation effects. Female. Follow-Up Studies. Hearing / drug effects. Hearing / physiology. Hearing / radiation effects. Humans. Infant. Magnetic Resonance Imaging / methods. Male. Outcome Assessment (Health Care) / methods. Radiotherapy / adverse effects. Time Factors. Verbal Learning / drug effects. Verbal Learning / physiology. Verbal Learning / radiation effects. Vision, Ocular / drug effects. Vision, Ocular / physiology. Vision, Ocular / radiation effects. Young Adult

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  • (PMID = 18974990.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
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5. Santagata S, Hornick JL, Ligon KL: Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG. Am J Surg Pathol; 2006 Dec;30(12):1613-8
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  • [Title] Comparative analysis of germ cell transcription factors in CNS germinoma reveals diagnostic utility of NANOG.
  • To assess the diagnostic utility of NANOG in central nervous system (CNS) germ cell tumors, we analyzed its expression by immunohistochemistry in a series of 12 CNS germinomas and compared its expression with other stem cell markers.
  • Strong nuclear expression of NANOG was demonstrated in >90% of the tumor cells in all cases.
  • NANOG was not detected in tumor types frequently considered in the differential diagnosis of CNS germinoma: pineoblastoma, primitive neuroectodermal tumors, medulloblastoma, lymphoma, pituitary adenoma, atypical teratoid/rhabdoid tumor, Langerhans cell histiocytosis, and gliomas.
  • These findings demonstrate that NANOG is a sensitive and specific marker of CNS germinoma.
  • These findings also suggest a potential biologic role for NANOG in maintenance of CNS germinoma.
  • [MeSH-major] Brain Neoplasms / metabolism. DNA-Binding Proteins / metabolism. Germinoma / metabolism. Homeodomain Proteins / metabolism
  • [MeSH-minor] Adolescent. Adult. Alkaline Phosphatase / metabolism. Biomarkers, Tumor / metabolism. Cell Nucleus / metabolism. Child. HMGB Proteins / metabolism. Humans. Isoenzymes / metabolism. Octamer Transcription Factor-3 / metabolism. SOXB1 Transcription Factors. Transcription Factors / metabolism

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  • (PMID = 17122519.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Grant] United States / NINDS NIH HHS / NS / NS047213
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / HMGB Proteins; 0 / Homeodomain Proteins; 0 / Isoenzymes; 0 / NANOG protein, human; 0 / Octamer Transcription Factor-3; 0 / POU5F1 protein, human; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / Transcription Factors; 0 / germ-cell AP isoenzyme; EC 3.1.3.1 / Alkaline Phosphatase
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6. Glassmann A, Molly S, Surchev L, Nazwar TA, Holst M, Hartmann W, Baader SL, Oberdick J, Pietsch T, Schilling K: Developmental expression and differentiation-related neuron-specific splicing of metastasis suppressor 1 (Mtss1) in normal and transformed cerebellar cells. BMC Dev Biol; 2007;7:111
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  • Given the prime importance of this pathway for cerebellar development and tumorigenesis, we assessed expression of Mtss1 in the developing murine cerebellum and human medulloblastoma specimens.
  • It is also expressed by granule cell precursor-derived medulloblastomas.
  • In the adult CNS, Mtss1 is found exclusively in cerebellar Purkinje cells.
  • Whereas immature granule cells express a Mtss1 variant observed also in peripheral tissues and comprising exon 12, this exon is replaced by a CNS-specific exon, 12a, in more mature granule cells and in adult Purkinje cells.
  • [MeSH-minor] Animals. Cerebellar Neoplasms / pathology. Exons. Humans. Mice. Mice, Inbred C57BL. Mice, Transgenic. Polymerase Chain Reaction. Protein Splicing / genetics. Purkinje Cells / pathology. Tumor Cells, Cultured

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  • (PMID = 17925019.001).
  • [ISSN] 1471-213X
  • [Journal-full-title] BMC developmental biology
  • [ISO-abbreviation] BMC Dev. Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Microfilament Proteins; 0 / Mtss1 protein, mouse; 0 / Neoplasm Proteins
  • [Other-IDs] NLM/ PMC2194783
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7. Pogoda JM, Preston-Martin S, Howe G, Lubin F, Mueller BA, Holly EA, Filippini G, Peris-Bonet R, McCredie MR, Cordier S, Choi W: An international case-control study of maternal diet during pregnancy and childhood brain tumor risk: a histology-specific analysis by food group. Ann Epidemiol; 2009 Mar;19(3):148-60
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  • [Title] An international case-control study of maternal diet during pregnancy and childhood brain tumor risk: a histology-specific analysis by food group.
  • PURPOSE: Maternal dietary data from an international collaborative case-control study on childhood brain tumors were used to evaluate associations between histology-specific risk and consumption of specific food groups during pregnancy.
  • Other histology-specific associations were decreased risk of anaplastic astrocytomas from cruciferous vegetables (OR, 0.4; 95% CI, 0.3-0.7 for 4th vs. 1st quartile; p trend<0.0001), decreased risk of astroglial tumors from fresh fish (OR, 0.6; 95% CI, 0.5-0.9 for 4th vs. 1st quartile; p trend=0.008), and increased risk of medulloblastoma from oil products (OR, 1.5; 95% CI, 1.0-2.2 for 4th vs. 1(st) quartile; p trend=0.005).
  • CONCLUSIONS: These results suggest the need for dietary analysis not only by brain tumor histology, but also by specific foods within a broad food group.

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  • (PMID = 19216997.001).
  • [ISSN] 1873-2585
  • [Journal-full-title] Annals of epidemiology
  • [ISO-abbreviation] Ann Epidemiol
  • [Language] ENG
  • [Grant] United States / NIEHS NIH HHS / ES / P30 ES007048; United States / NCI NIH HHS / CA / P01 CA017054-30; United States / NCI NIH HHS / PC / N01 PC035139; United States / NCI NIH HHS / CA / CA47082; United States / NIEHS NIH HHS / ES / P30 ES007048-029003; United States / NCI NIH HHS / CA / CA47081; United States / NCI NIH HHS / CA / CA17054; United States / NCI NIH HHS / CN / N01-CN-05230; United States / NIEHS NIH HHS / ES / ES007048-029003; United States / NCI NIH HHS / CN / N01-CN-25403; United States / NCI NIH HHS / CA / P01 CA017054; United States / NCI NIH HHS / CA / N01 CN025403; United States / NCI NIH HHS / CN / N01 CN005230; United States / NIEHS NIH HHS / ES / 5P30 ES07048
  • [Publication-type] Journal Article; Multicenter Study; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Nitroso Compounds
  • [Other-IDs] NLM/ NIHMS98974; NLM/ PMC2832584
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8. Pierson J, Hostager B, Fan R, Vibhakar R: Regulation of cyclin dependent kinase 6 by microRNA 124 in medulloblastoma. J Neurooncol; 2008 Oct;90(1):1-7
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  • [Title] Regulation of cyclin dependent kinase 6 by microRNA 124 in medulloblastoma.
  • Despite recent advances in treatment medulloblastoma continues to remain a vexing problem.
  • Recently increased expression of cyclin dependent kinase 6 (CDK6) was identified as an adverse prognostic marker in medulloblastoma.
  • We hypothesized that CDK6 expression is also regulated by microRNAs in medulloblastoma.
  • We identified putative miR sites in the CDK6 including microRNA 124a, a brain enriched microRNA.
  • Expression of miR 124a was significantly decreased in medulloblastoma cells compared to normal adult cerebellum.
  • Functional association between miR 124a and CDK6 in medulloblastoma was established using luciferase assays.
  • Additionally, re-expression of miR 124a in medulloblastoma cells decreased expression of CDK6 protein.
  • Transfection of miR 124 significantly decreases medulloblastoma cell growth but does not alter apoptosis.
  • Our data strongly indicate that CDK6 is regulated by microRNA 124 in medulloblastoma and that miR 124 modulates medulloblastoma cell growth.
  • [MeSH-major] Brain Neoplasms / genetics. Cyclin-Dependent Kinase 6 / genetics. Gene Expression Regulation, Neoplastic. Medulloblastoma / genetics. MicroRNAs / genetics
  • [MeSH-minor] Apoptosis / physiology. Base Sequence. Blotting, Western. Cell Line, Tumor. Cell Proliferation. Humans. Molecular Sequence Data. RNA, Messenger / analysis. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 18607543.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / MicroRNAs; 0 / RNA, Messenger; EC 2.7.11.22 / Cyclin-Dependent Kinase 6
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9. Menon G, Krishnakumar K, Nair S: Adult medulloblastoma: clinical profile and treatment results of 18 patients. J Clin Neurosci; 2008 Feb;15(2):122-6
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  • [Title] Adult medulloblastoma: clinical profile and treatment results of 18 patients.
  • The objective of this article is to examine the clinicoradiological features and surgical outcomes of adult patients (>16 years) with medulloblastoma.
  • An attempt was made to identify the predictors of poor outcome and assess patterns of relapse and to compare these with pediatric medulloblastoma.
  • Retrospective case record analyses were performed on 18 adults (>16 years) and 79 children (<16 years) operated upon after January 1990, who had at least 5 years of follow-up.
  • The tumor was located in the vermis in 12 patients (66.6%) and in the cerebellar hemisphere in six (16.6%).
  • In spite of recent advances in management, patients with medulloblastoma still have a poor prognosis.
  • However, adults fared better than children.
  • Vermian location had a better outcome in adults, but not in children.
  • Desmoplastic variant histology was not observed to be a significant prognostic factor in the adult group while brain stem invasion carried a poor prognosis.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Clinical Trials as Topic. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Female. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Statistics, Nonparametric. Treatment Outcome

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  • (PMID = 18078755.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
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10. Mühlisch J, Bajanowski T, Rickert CH, Roggendorf W, Würthwein G, Jürgens H, Frühwald MC: Frequent but borderline methylation of p16 (INK4a) and TIMP3 in medulloblastoma and sPNET revealed by quantitative analyses. J Neurooncol; 2007 May;83(1):17-29
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  • [Title] Frequent but borderline methylation of p16 (INK4a) and TIMP3 in medulloblastoma and sPNET revealed by quantitative analyses.
  • Certain risk groups among tumors of the central nervous system (CNS) in children take an almost inevitably fatal course.
  • Aberrant methylation is common in malignant brain tumors of childhood and may have implications for stratification and therapy.
  • Methylation of p16 (INK4A), p14 (ARF), TIMP3, CDH1, p15 (INK4B )and DAPK1 in medulloblastoma (MB) and ependymoma has been discussed controversially in the literature.
  • Only p16 (INK4A )and TIMP3 were methylated consistently in medulloblastomas (p16 (INK4A ) 14%, TIMP3 11%) and p16 (INK4A) also in anaplastic ependymomas (1/4 tumors).
  • Therapeutic and diagnostic implications urge into depth analyses of methylation as a mechanism, which might fill some of the gaps of our understanding of brain tumor origin.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. DNA Methylation. Genes, p16. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Tissue Inhibitor of Metalloproteinase-3 / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Apoptosis Regulatory Proteins / genetics. Calcium-Calmodulin-Dependent Protein Kinases / genetics. Child. Child, Preschool. Death-Associated Protein Kinases. Female. Gene Silencing. Humans. Infant. Male. Middle Aged. Nerve Tissue Proteins / genetics. Receptors, Immunologic / genetics

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  • (PMID = 17206475.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / Nerve Tissue Proteins; 0 / Receptors, Immunologic; 0 / TIMP3 protein, human; 0 / Tissue Inhibitor of Metalloproteinase-3; 0 / roundabout protein; EC 2.7.11.1 / DAPK1 protein, human; EC 2.7.11.1 / Death-Associated Protein Kinases; EC 2.7.11.17 / Calcium-Calmodulin-Dependent Protein Kinases
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11. Shu XH, Li H, Sun Z, Wu ML, Ma JX, Wang JM, Wang Q, Sun Y, Fu YS, Chen XY, Kong QY, Liu J: Identification of metabolic pattern and bioactive form of resveratrol in human medulloblastoma cells. Biochem Pharmacol; 2010 May 15;79(10):1516-25
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  • [Title] Identification of metabolic pattern and bioactive form of resveratrol in human medulloblastoma cells.
  • This issue was addressed here by identifying the metabolic pattern and the bioactive form of resveratrol in a resveratrol-sensitive human medulloblastoma cell line, UW228-3.
  • The cell lysates and condition media of UW228-3 cells with or without 100 microM resveratrol treatment were analyzed by HPLC and LC/MS which revealed (1) that resveratrol was chemically unstable and the spontaneous generation of cis-resveratrol reduced resveratrol's anti-medulloblastoma efficacy and (2) that resveratrol monosulfate was the major metabolite of the cells.
  • To identify the bioactive form of resveratrol, a mixture-containing approximately half fraction of resveratrol monosulfate was prepared by incubating trans-resveratrol with freshly prepared rat brain lysates.
  • Medulloblastoma cells treated by 100 microM of this mixture showed attenuated cell crisis.
  • The overall levels of the three brain-associated sulfotransferases (SULT1A1, 1C2 and 4A1) were low in medulloblastoma cells in vivo and in vitro in comparison with that in human noncancerous and rat normal cerebella; resveratrol could more or less up-regulate the production of these enzymes in UW228-3 cells but their overall level was still lower than that in normal cerebellum tissue.
  • Our study thus demonstrated for the first time that trans-resveratrol is the bioactive form in medulloblastoma cells in which the expression of brain-associated SULTs was down-regulated, resulting in the increased intracellular bioavailability and anti-medulloblastoma efficacy of trans-resveratrol.
  • [MeSH-major] Antineoplastic Agents / metabolism. Antineoplastic Agents / pharmacokinetics. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy. Stilbenes / pharmacokinetics
  • [MeSH-minor] Adolescent. Animals. Biotransformation. Blotting, Western. Cell Line, Tumor. Child. Chromatography, High Pressure Liquid. Humans. Rats. Reverse Transcriptase Polymerase Chain Reaction. Sulfotransferases / metabolism. Young Adult

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  • [Copyright] 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20105429.001).
  • [ISSN] 1873-2968
  • [Journal-full-title] Biochemical pharmacology
  • [ISO-abbreviation] Biochem. Pharmacol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Stilbenes; EC 2.8.2.- / Sulfotransferases; Q369O8926L / resveratrol
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12. Sousa R, Sá G, Reimão S, Lopes L, Ruivo J, Albuquerque L, Campos J: [Adult cerebellar medulloblastoma: imaging findings in eight cases]. Acta Med Port; 2006 Nov-Dec;19(6):466-70
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  • [Title] [Adult cerebellar medulloblastoma: imaging findings in eight cases].
  • Medulloblastoma is a brain tumor of neuroepithelial origin, frequent in children but rare in adults.
  • The imaging pattern is well studied in the pediatric group thought there is controversy about the imaging characteristics in adults.
  • We report CT and MRI imaging findings of 8 adult patients with cerebellar medulloblastoma.
  • The imaging findings of medulloblastomas in adults are unspecific and different from those in child.
  • They should be considered in the differential diagnosis of cerebellar tumor in adults, especially if they are hyperdense on CT, with well defined margins, with superficial extension and with dural involvement.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Cerebellar Neoplasms / radiography. Medulloblastoma / pathology. Medulloblastoma / radiography
  • [MeSH-minor] Adolescent. Adult. Cerebellum / pathology. Cerebellum / radiography. Female. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 17583605.001).
  • [ISSN] 1646-0758
  • [Journal-full-title] Acta médica portuguesa
  • [ISO-abbreviation] Acta Med Port
  • [Language] por
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Portugal
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13. Puri S, Joshi BH, Sarkar C, Mahapatra AK, Hussain E, Sinha S: Expression and structure of interleukin 4 receptors in primary meningeal tumors. Cancer; 2005 May 15;103(10):2132-42
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  • BACKGROUND: It was reported previously that malignant human tumors, like glioma and medulloblastoma, express high-density interleukin (IL-4) receptor mRNA and protein.
  • Because IL-4 receptors (R) are sensitive targets for targeted therapeutics, knowledge of the expression of these receptors in other central nervous system tumors is of great interest.
  • METHODS: Reverse transcription-polymerase chain reaction (RT-PCR) analysis for IL-13Ralpha1, IL-4Ralpha and IL-2Rgammac was performed on total RNA extracted from 35 meningiomas and a normal human brain tissue sample.
  • RESULTS: Transcripts for the IL-4Ralpha and IL-13Ralpha1 chains were overexpressed in meningiomas compared with normal brain tissue.
  • The transcripts for IL-2Rgammac chain were not detected in any of the tumor samples or in normal brain tissue.
  • [MeSH-minor] Adult. Age Factors. Brain Chemistry. Fluorescent Antibody Technique. Gene Expression Regulation, Neoplastic. Humans. Interleukin Receptor Common gamma Subunit. Interleukin-13 / chemistry. Interleukin-13 Receptor alpha1 Subunit. Middle Aged. RNA, Messenger / analysis. Receptors, Interleukin / chemistry. Receptors, Interleukin-13. Receptors, Interleukin-2 / chemistry. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15830341.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / IL13RA1 protein, human; 0 / IL2RG protein, human; 0 / Interleukin Receptor Common gamma Subunit; 0 / Interleukin-13; 0 / Interleukin-13 Receptor alpha1 Subunit; 0 / RNA, Messenger; 0 / Receptors, Interleukin; 0 / Receptors, Interleukin-13; 0 / Receptors, Interleukin-2; 0 / Receptors, Interleukin-4
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14. Poelen J, Bernsen HJ, Prick MJ: Metastatic medulloblastoma in an adult; treatment with temozolomide. Acta Neurol Belg; 2007 Jun;107(2):51-4
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  • [Title] Metastatic medulloblastoma in an adult; treatment with temozolomide.
  • Medulloblastoma is a malignant brain tumour most frequently seen in children.
  • Relapses of medulloblastoma are sensitive to chemotherapy and treatment with chemotherapeutics in children has increased the survival rates.
  • A medulloblastoma at adult age is extremely rare, and there is no overall accepted treatment, especially not in the case of a relapse.
  • This observation encouraged us to decide to treat an adult patient with a recurrent medulloblastoma with temozolomide.
  • This female patient showed a recurrence of a medulloblastoma 7 years after the initial presentation with metastatic spread along the neuraxis and progressive neurological deterioration.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Cerebellar Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Medulloblastoma / drug therapy. Neoplasm Recurrence, Local / drug therapy
  • [MeSH-minor] Adult. Female. Humans. Magnetic Resonance Imaging. Spinal Cord Neoplasms / drug therapy. Spinal Cord Neoplasms / secondary

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  • (PMID = 17710841.001).
  • [ISSN] 0300-9009
  • [Journal-full-title] Acta neurologica Belgica
  • [ISO-abbreviation] Acta Neurol Belg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Belgium
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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15. Phi JH, Kim JH, Eun KM, Wang KC, Park KH, Choi SA, Kim YY, Park SH, Cho BK, Kim SK: Upregulation of SOX2, NOTCH1, and ID1 in supratentorial primitive neuroectodermal tumors: a distinct differentiation pattern from that of medulloblastomas. J Neurosurg Pediatr; 2010 Jun;5(6):608-14
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  • [Title] Upregulation of SOX2, NOTCH1, and ID1 in supratentorial primitive neuroectodermal tumors: a distinct differentiation pattern from that of medulloblastomas.
  • OBJECT: Supratentorial primitive neuroectodermal tumor (PNET) and medulloblastoma are highly malignant embryonal brain tumors.
  • The authors compared the expression of specific genes involved in neuroglial differentiation in supratentorial PNETs and medulloblastomas to define the distinct characters of these tumors.
  • METHODS: The mRNA expression of 8 genes (SOX2, NOTCH1, ID1, ASCL-1, NEUROD1, NEUROG1, NEUROG2, and NRG1) was evaluated in 25 embryonal tumors (12 supratentorial PNETs and 13 medulloblastomas) by quantitative real-time polymerase chain reaction.
  • The expression levels of the transcripts of these genes were compared between the tumor groups.
  • RESULTS: Supratentorial PNETs expressed significantly higher levels of SOX2, NOTCH1, ID1, and ASCL-1 transcripts, whereas the transcription of proneural basic helix-loop-helix factors, NEUROD1, NEUROG1 (significantly), and NEUROG2 (not significantly) was upregulated in medulloblastomas.
  • The proportion of phosphorylated STAT3alpha relative to STAT3alpha was significantly greater in supratentorial PNETs than in medulloblastomas, indicating activation of the JAK/STAT3 pathway in supratentorial PNETs.
  • CONCLUSIONS: These results indicate that supratentorial PNET predominantly has glial features and medulloblastoma largely follows a neuronal differentiation pattern.
  • These divergent differentiation patterns may be related to the location and origin of each tumor.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Inhibitor of Differentiation Protein 1 / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Receptor, Notch1 / genetics. SOXB1 Transcription Factors / genetics. STAT3 Transcription Factor / genetics. Supratentorial Neoplasms / genetics. Up-Regulation / genetics
  • [MeSH-minor] Adolescent. Adult. Cerebellum / pathology. Cerebral Cortex / pathology. Child. Child, Preschool. Diagnosis, Differential. Female. Gene Expression Regulation, Neoplastic / genetics. Humans. Infant. Infant, Newborn. Male. Neuroglia / pathology. Neurons / pathology. RNA, Messenger / genetics. Reverse Transcriptase Polymerase Chain Reaction. Transcription, Genetic / genetics

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  • (PMID = 20515335.001).
  • [ISSN] 1933-0715
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ID1 protein, human; 0 / Inhibitor of Differentiation Protein 1; 0 / NOTCH1 protein, human; 0 / RNA, Messenger; 0 / Receptor, Notch1; 0 / SOX2 protein, human; 0 / SOXB1 Transcription Factors; 0 / STAT3 Transcription Factor; 0 / STAT3 protein, human
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16. Lai R: Survival of patients with adult medulloblastoma: a population-based study. Cancer; 2008 Apr 1;112(7):1568-74
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  • [Title] Survival of patients with adult medulloblastoma: a population-based study.
  • BACKGROUND: Adult medulloblastoma accounts for less than 1% of adult intracranial tumors.
  • RESULTS: Four hundred fifty-four patients with adult medulloblastoma were diagnosed from 1973-2004 in the 17 regions covered by SEER.
  • [MeSH-major] Cerebellar Neoplasms / mortality. Medulloblastoma / mortality
  • [MeSH-minor] Adult. Cohort Studies. Female. Follow-Up Studies. Humans. Male. Prognosis. Registries. SEER Program. Survival Rate

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  • (PMID = 18278809.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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17. Sugita Y, Nakamura Y, Yamamoto M, Ogasawara S, Ohshima K, Shigemori M: Expression of KIAA 0864 protein in neuroepithelial tumors: an analysis based on the presence of monoclonal antibody HFB-16. J Neurooncol; 2008 Sep;89(2):151-8
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  • The KIAA 0864 (KA) protein is a putative protein of a cDNA from 100 cDNA clones that was newly determined from a set of size-fractionated human brain cDNA libraries and their coding potentials of large proteins (180-200 kD) by using in vitro transcription assays.
  • Among the 55 NETs, a moderate-to-intense KA protein immunoreactivity was observed in 8 of 8 medulloblastomas, 1 of 1 central nervous system supratentorial primitive neuroectodermal tumor (CNS supratentorial PNET), 4 of 4 retinoblastomas, 1 of 1 neuroblastoma, 8 of 8 central neurocytomas, 4 of 4 oligodendrogliomas, 4 of 4 oligoastrocytomas, 1 of 1 extraventricular neurocytoma, and 1 of 1 gangliocytoma.
  • In addition, it could be a useful tool for performing the differential diagnosis between GBs and CNS supratentorial PNET.
  • [MeSH-major] Cerebellar Neoplasms / metabolism. Guanine Nucleotide Exchange Factors / metabolism. Medulloblastoma / metabolism. Neoplasms, Neuroepithelial / metabolism
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antibodies, Monoclonal. Child. Child, Preschool. Female. Humans. Male. Middle Aged. Retrospective Studies

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  • (PMID = 18458818.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Guanine Nucleotide Exchange Factors; 0 / RASGRP3 protein, human
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18. Lasky JL 3rd, Choe M, Nakano I: Cancer stem cells in pediatric brain tumors. Curr Stem Cell Res Ther; 2009 Dec;4(4):298-305
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  • [Title] Cancer stem cells in pediatric brain tumors.
  • Central nervous system (CNS) tumors remain the leading cause of death among pediatric neoplasms.
  • Although standard therapies cure many pediatric CNS tumors, the long-term cognitive and physical consequences of these therapies are devastating.
  • Although recent studies have focused on molecular mechanisms that underlie the initiation and progression of adult glioblastoma multiforme (GBM), these tumors differ phenotypically and at a molecular level from pediatric brain tumors.
  • Recent investigations have identified a stem cell population, termed "brain tumor stem cells" (BTSC) within the heterogeneous cell populations that comprise malignant brain tumors which may be partly responsible for the resistance to current therapies.
  • These have been identified in several pediatric tumors including medulloblastoma, ependymomas, and malignant gliomas.
  • By exploiting molecular differences present within these heterogeneous populations of brain tumor cells, we may be able to achieve specific eradication of BTSC and long-lasting remissions, while causing less toxicity to normal tissues.
  • In this review, we describe the issues surrounding the identification and characterization of BTSC, the molecular biology of BTSC for different pediatric brain tumors, and suggest future avenues for the development of treatments for this devastating disease.
  • [MeSH-major] Brain Neoplasms / pathology. Ependymoma / pathology. Medulloblastoma / pathology. Neoplastic Stem Cells / pathology. Optic Nerve Glioma / pathology
  • [MeSH-minor] Adult Stem Cells / pathology. Biomarkers / metabolism. Cell Differentiation. Chemotherapy, Adjuvant. Child. Humans. Surgical Procedures, Operative


19. Wendling DS, Lück C, von Schweinitz D, Kappler R: Characteristic overexpression of the forkhead box transcription factor Foxf1 in Patched-associated tumors. Int J Mol Med; 2008 Dec;22(6):787-92
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  • Patients with nevoid basal cell carcinoma syndrome carry germline mutations in the tumor suppressor gene Patched 1 (PTCH1) and are predisposed to develop basal cell carcinoma (BCC), medulloblastoma (MB), and rhabdomyosarcoma (RMS).
  • In contrast, basal expression levels of Foxf1, Gli1, Bmi1 and Notch2 were detected in a variety of adult mouse tissues, such as liver, kidney, spleen, lung, heart and brain.
  • [MeSH-major] Carcinoma, Basal Cell / metabolism. Forkhead Transcription Factors / metabolism. Medulloblastoma / metabolism. Receptors, Cell Surface / genetics. Signal Transduction / physiology

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  • (PMID = 19020777.001).
  • [ISSN] 1107-3756
  • [Journal-full-title] International journal of molecular medicine
  • [ISO-abbreviation] Int. J. Mol. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Actins; 0 / Bmi1 protein, mouse; 0 / Forkhead Transcription Factors; 0 / Foxf1a protein, mouse; 0 / Gli protein, mouse; 0 / Hedgehog Proteins; 0 / Kruppel-Like Transcription Factors; 0 / Notch2 protein, mouse; 0 / Nuclear Proteins; 0 / Proto-Oncogene Proteins; 0 / Receptor, Notch2; 0 / Receptors, Cell Surface; 0 / Repressor Proteins; 0 / patched receptors; EC 6.3.2.19 / Polycomb Repressive Complex 1
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20. Koontz NA, Hess CP: AJR teaching file: brain tumor in a patient with familial adenomatous polyposis. AJR Am J Roentgenol; 2010 Sep;195(3 Suppl):S25-8
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  • [Title] AJR teaching file: brain tumor in a patient with familial adenomatous polyposis.
  • [MeSH-major] Adenomatous Polyposis Coli / complications. Brain Neoplasms / complications. Brain Neoplasms / diagnosis. Magnetic Resonance Imaging. Medulloblastoma / complications. Medulloblastoma / diagnosis
  • [MeSH-minor] Adult. Diagnosis, Differential. Female. Humans


21. Warren K, Jakacki R, Widemann B, Aikin A, Libucha M, Packer R, Vezina G, Reaman G, Shaw D, Krailo M, Osborne C, Cehelsky J, Caldwell D, Stanwood J, Steinberg SM, Balis FM: Phase II trial of intravenous lobradimil and carboplatin in childhood brain tumors: a report from the Children's Oncology Group. Cancer Chemother Pharmacol; 2006 Sep;58(3):343-7
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  • [Title] Phase II trial of intravenous lobradimil and carboplatin in childhood brain tumors: a report from the Children's Oncology Group.
  • BACKGROUND: [corrected] Lobradimil is a synthetic bradykinin analog that rapidly and transiently increases the permeability of the blood-brain barrier (BBB).
  • The combination of lobradimil and carboplatin was studied in pediatric patients with primary brain tumors in a phase II trial, the primary endpoints of which were to estimate the response rate and time to disease progression.
  • PATIENTS AND METHODS: Patients were stratified by histology into five cohorts: brainstem glioma, high-grade glioma, low-grade glioma, medullobastoma/primitive neuroectodermal tumor (PNET), and ependymoma.
  • The study was closed for commercial reasons prior to achieving the accrual goals for the ependymoma (n = 8), medulloblastoma/PNET (n = 6) and low-grade glioma (n = 2) cohorts, although responses were observed in 1 patient with PNET and 2 patients with ependymoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Blood-Brain Barrier / metabolism. Brain Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Bradykinin / administration & dosage. Bradykinin / adverse effects. Bradykinin / analogs & derivatives. Bradykinin / therapeutic use. Carboplatin / administration & dosage. Carboplatin / adverse effects. Carboplatin / therapeutic use. Child. Child, Preschool. Cohort Studies. Drug Administration Schedule. Humans. Infusions, Intravenous. Treatment Outcome

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  • (PMID = 16408203.001).
  • [ISSN] 0344-5704
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Grant] United States / Intramural NIH HHS / /
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 159768-75-9 / RMP 7; BG3F62OND5 / Carboplatin; S8TIM42R2W / Bradykinin
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22. Spreafico F, Massimino M, Gandola L, Cefalo G, Mazza E, Landonio G, Pignoli E, Poggi G, Terenziani M, Pedrazzoli P, Siena S, Fossati-Bellani F: Survival of adults treated for medulloblastoma using paediatric protocols. Eur J Cancer; 2005 Jun;41(9):1304-10
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  • [Title] Survival of adults treated for medulloblastoma using paediatric protocols.
  • We retrospectively studied 26 consecutive adults treated for medulloblastoma using paediatric protocols.
  • Although the number of patients is limited, our data suggest that the sandwich sequential, moderately intensive chemotherapy in combination with HART is an effective treatment for medulloblastoma in adults, and this approach seems to overcome previously-recognised risk factors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Chemotherapy, Adjuvant / methods. Cranial Irradiation / methods. Disease-Free Survival. Humans. Infusions, Intravenous. Lomustine / administration & dosage. Lomustine / adverse effects. Methotrexate / administration & dosage. Methotrexate / adverse effects. Middle Aged. Neoplasm Recurrence, Local / etiology. Neoplasm Recurrence, Local / mortality. Retrospective Studies. Treatment Outcome. Vincristine / administration & dosage. Vincristine / adverse effects

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  • (PMID = 15869875.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine; YL5FZ2Y5U1 / Methotrexate
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23. McCabe MG, Ichimura K, Liu L, Plant K, Bäcklund LM, Pearson DM, Collins VP: High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors. J Neuropathol Exp Neurol; 2006 Jun;65(6):549-61
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  • [Title] High-resolution array-based comparative genomic hybridization of medulloblastomas and supratentorial primitive neuroectodermal tumors.
  • Medulloblastomas and supratentorial primitive neuroectodermal tumors are aggressive childhood tumors.
  • We report our findings using array comparative genomic hybridization (CGH) on a whole-genome BAC/PAC/cosmid array with a median clone separation of 0.97 Mb to study 34 medulloblastomas and 7 supratentorial primitive neuroectodermal tumors.
  • In addition, one supratentorial primitive neuroectodermal tumor had lost both copies of the tumor-suppressor genes CDKN2A and CDKN2B.
  • Ten medulloblastomas had findings suggestive of isochromosome 17q.
  • In contrast to previous reports using conventional CGH, array CGH identified 3 distinct breakpoints in these cases: Ch 17: 17940393-19251679 (17p11.2, n = 6), Ch 17: 20111990-23308272 (17p11.2-17q11.2, n = 4), and Ch 17: 38425359-39091575 (17q21.31, n = 1).
  • Significant differences were found in the patterns of copy number change between medulloblastomas and supratentorial primitive neuroectodermal tumors, providing further evidence that these tumors are genetically distinct despite their morphologic and behavioral similarities.
  • [MeSH-major] Brain Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Nucleic Acid Hybridization / methods. Supratentorial Neoplasms / genetics
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Chromosome Aberrations. Gene Expression Profiling. Humans. In Situ Hybridization, Fluorescence / methods. Infant. Reverse Transcriptase Polymerase Chain Reaction / methods

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  • (PMID = 16783165.001).
  • [ISSN] 0022-3069
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / / A6618
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2816352; NLM/ UKMS2695
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24. Brandes AA, Franceschi E, Tosoni A, Blatt V, Ermani M: Long-term results of a prospective study on the treatment of medulloblastoma in adults. Cancer; 2007 Nov 1;110(9):2035-41
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  • [Title] Long-term results of a prospective study on the treatment of medulloblastoma in adults.
  • BACKGROUND: Because medulloblastoma (MB) is rare in adults, the few studies on this condition have been retrospective, and the follow-up has tended to be short.
  • METHODS: In 1989, a prospective Phase II trial was initiated to evaluate the efficacy of treatment for adults with MB.
  • Patients were staged completely with a neuroradiologic examination of the brain and neuroaxis and by cerebrospinal fluid cytology, according to Chang's staging system.
  • RESULTS: After a median follow up of 7.6 years, among a total of 36 adults with MB, the overall progression-free survival (PFS) and overall survival (OS) rates at 5 years were 72% and 75%, respectively.
  • CONCLUSIONS: In adult patients with MB, long-term follow-up was essential for evaluating the real impact of treatments.
  • [MeSH-major] Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiotherapy. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Combined Modality Therapy. Disease-Free Survival. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local / epidemiology. Neoplasm Recurrence, Local / mortality. Neoplasm Recurrence, Local / therapy. Neoplasm Staging. Prospective Studies. Radiotherapy. Survival Rate. Time. Treatment Outcome

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  • (PMID = 17823910.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article
  • [Publication-country] United States
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25. Perry A, Miller CR, Gujrati M, Scheithauer BW, Zambrano SC, Jost SC, Raghavan R, Qian J, Cochran EJ, Huse JT, Holland EC, Burger PC, Rosenblum MK: Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases. Brain Pathol; 2009 Jan;19(1):81-90
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  • [Title] Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases.
  • Central nervous system neoplasms with combined features of malignant glioma and primitive neuroectodermal tumor (MG-PNET) are rare, poorly characterized, and pose diagnostic as well as treatment dilemmas.
  • Anaplasia, as seen in medulloblastomas, was noted in 70%.
  • (ii) may represent a metaplastic process or expansion of a tumor stem/progenitor cell clone;.
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / genetics. Brain Neoplasms / pathology. Brain Neoplasms / therapy. Combined Modality Therapy. Dacarbazine / analogs & derivatives. Dacarbazine / therapeutic use. Female. Follow-Up Studies. Genes, myc / genetics. Humans. In Situ Hybridization, Fluorescence. Medulloblastoma / genetics. Medulloblastoma / pathology. Medulloblastoma / therapy. Neoplasm Metastasis. Neoplasms, Multiple Primary / genetics. Neoplasms, Multiple Primary / pathology. Neoplasms, Multiple Primary / therapy. Prognosis. Radiotherapy / methods. Treatment Outcome. Young Adult

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  • (PMID = 18452568.001).
  • [ISSN] 1750-3639
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 0 / Proto-Oncogene Proteins; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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26. Padovani L, Sunyach MP, Perol D, Mercier C, Alapetite C, Haie-Meder C, Hoffstetter S, Muracciole X, Kerr C, Wagner JP, Lagrange JL, Maire JP, Cowen D, Frappaz D, Carrie C: Common strategy for adult and pediatric medulloblastoma: a multicenter series of 253 adults. Int J Radiat Oncol Biol Phys; 2007 Jun 1;68(2):433-40
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  • [Title] Common strategy for adult and pediatric medulloblastoma: a multicenter series of 253 adults.
  • PURPOSE: To assess prognostic factors for adults with medulloblastoma in a multicenter, retrospective study.
  • METHODS AND MATERIALS: Data were collected by file review or mail inquiry for 253 adults treated between 1975 to 2004.
  • CONCLUSION: We report the largest series of medulloblastoma in adults.
  • [MeSH-major] Cerebellar Neoplasms / drug therapy. Cerebellar Neoplasms / radiotherapy. Medulloblastoma / drug therapy. Medulloblastoma / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Age Factors. Analysis of Variance. Combined Modality Therapy / methods. Female. Follow-Up Studies. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Survival Rate

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  • (PMID = 17498567.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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27. Zitterbart K, Zavrelova I, Kadlecova J, Spesna R, Kratochvilova A, Pavelka Z, Sterba J: p73 expression in medulloblastoma: TAp73/DeltaNp73 transcript detection and possible association of p73alpha/DeltaNp73 immunoreactivity with survival. Acta Neuropathol; 2007 Dec;114(6):641-50
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  • [Title] p73 expression in medulloblastoma: TAp73/DeltaNp73 transcript detection and possible association of p73alpha/DeltaNp73 immunoreactivity with survival.
  • Recently, several TP73 transcripts have been revealed in medulloblastoma (MB), the most common malignant brain tumor in children.
  • We report significant differences for TAp73 and DeltaNp73 mRNA expression between tumor tissues and reference (P = 0.013, P = 0.028).
  • In normal cerebellum, positive staining for p73alpha and DeltaNp73 was observed in the Purkinje cells of newborns, not adult samples, which supports the developmental role of TP73 during organogenesis of the human cerebellum.
  • Our results indicate the involvement of p73 protein in MB tumorigenesis and define TP73 as a potential prognostic and therapeutic target for medulloblastoma.
  • [MeSH-major] Brain Neoplasms / metabolism. DNA-Binding Proteins / metabolism. Medulloblastoma / metabolism. Nuclear Proteins / metabolism. Tumor Suppressor Proteins / metabolism
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / analysis. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Brain / metabolism. Brain / pathology. Brain / physiopathology. Cell Transformation, Neoplastic / genetics. Cell Transformation, Neoplastic / metabolism. Child. Child, Preschool. Female. Gene Expression Regulation, Neoplastic / genetics. Humans. Immunohistochemistry. Male. Prognosis. Protein Isoforms / genetics. Protein Isoforms / isolation & purification. Protein Isoforms / metabolism. RNA, Messenger / analysis. RNA, Messenger / metabolism. Retrospective Studies. Survival Rate

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  • [ErratumIn] Acta Neuropathol. 2008 Nov;116(5):579-80
  • (PMID = 17912537.001).
  • [ISSN] 0001-6322
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / DNA-Binding Proteins; 0 / Nuclear Proteins; 0 / Protein Isoforms; 0 / RNA, Messenger; 0 / Tumor Suppressor Proteins
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28. Schiebel P, Stippich C, Unterberg A: [Adult medulloblastoma]. Rofo; 2010 Sep;182(9):808-9
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  • [Title] [Adult medulloblastoma].
  • [Transliterated title] Adultes Medulloblastom.
  • [MeSH-major] Cerebellar Neoplasms / diagnosis. Diffusion Magnetic Resonance Imaging. Image Processing, Computer-Assisted. Magnetic Resonance Imaging. Medulloblastoma / diagnosis
  • [MeSH-minor] Adult. Cerebellum / pathology. Cerebellum / surgery. Contrast Media / administration & dosage. Craniotomy. Diagnosis, Differential. Humans. Male

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  • (PMID = 20544581.001).
  • [ISSN] 1438-9010
  • [Journal-full-title] RöFo : Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
  • [ISO-abbreviation] Rofo
  • [Language] ger
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Contrast Media
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29. Massimino M, Gandola L, Spreafico F, Biassoni V, Luksch R, Collini P, Solero CN, Simonetti F, Pignoli E, Cefalo G, Poggi G, Modena P, Mariani L, Potepan P, Podda M, Casanova M, Pecori E, Acerno S, Ferrari A, Terenziani M, Meazza C, Polastri D, Ravagnani F, Fossati-Bellani F: No salvage using high-dose chemotherapy plus/minus reirradiation for relapsing previously irradiated medulloblastoma. Int J Radiat Oncol Biol Phys; 2009 Apr 1;73(5):1358-63
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  • [Title] No salvage using high-dose chemotherapy plus/minus reirradiation for relapsing previously irradiated medulloblastoma.
  • PURPOSE: Myeloablative regimens were frequently used for medulloblastoma relapsing after craniospinal irradiation (CSI): in 1997-2002, we used repeated surgery, standard-dose and myeloablative chemotherapy, and reirradiation.
  • RESULTS: Seventeen patients were treated: previous treatment included CSI of 19.5-36 Gy with posterior fossa/tumor boost and chemotherapy in 16 patients.
  • Relapse sites included leptomeninges in 9 patients, spine in 4 patients, posterior fossa in 3 patients, and brain in 1 patient.
  • Twelve of 14 patients with assessable tumor had an objective response after reinduction; 2 experienced progression and were not given the myeloablative courses.
  • A salvage therapy for medulloblastoma after CSI still needs to be sought.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms. Medulloblastoma. Neoplasm Recurrence, Local. Salvage Therapy
  • [MeSH-minor] Adolescent. Carboplatin / administration & dosage. Carboplatin / adverse effects. Child. Child, Preschool. Cisplatin / administration & dosage. Cisplatin / adverse effects. Combined Modality Therapy / methods. Cyclophosphamide / administration & dosage. Cyclophosphamide / adverse effects. Disease-Free Survival. Drug Administration Schedule. Etoposide / administration & dosage. Etoposide / adverse effects. Female. Granulocyte Colony-Stimulating Factor / administration & dosage. Humans. Male. Methotrexate / administration & dosage. Methotrexate / adverse effects. Radiotherapy Dosage. Remission Induction / methods. Thiotepa / administration & dosage. Thiotepa / adverse effects. Vincristine / administration & dosage. Vincristine / adverse effects. Young Adult

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  • (PMID = 19019566.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 143011-72-7 / Granulocyte Colony-Stimulating Factor; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; 905Z5W3GKH / Thiotepa; BG3F62OND5 / Carboplatin; Q20Q21Q62J / Cisplatin; YL5FZ2Y5U1 / Methotrexate
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30. Franceschi E, Tosoni A, Paioli A, Cavallo G, Spagnolli F, Brandes AA: Challenges and progress in the treatment of adult medulloblastomas. Future Oncol; 2007 Apr;3(2):115-7
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  • [Title] Challenges and progress in the treatment of adult medulloblastomas.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Medulloblastoma / therapy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant / trends. Humans. Risk Factors

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  • (PMID = 17381408.001).
  • [ISSN] 1479-6694
  • [Journal-full-title] Future oncology (London, England)
  • [ISO-abbreviation] Future Oncol
  • [Language] eng
  • [Publication-type] Editorial
  • [Publication-country] England
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31. Kremer P, Fardanesh M, Ding R, Pritsch M, Zoubaa S, Frei E: Intraoperative fluorescence staining of malignant brain tumors using 5-aminofluorescein-labeled albumin. Neurosurgery; 2009 Mar;64(3 Suppl):ons53-60; discussion ons60-1
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  • [Title] Intraoperative fluorescence staining of malignant brain tumors using 5-aminofluorescein-labeled albumin.
  • OBJECTIVE: The newly developed conjugate 5-aminofluorescein (AFL)-human serum albumin (HSA) was investigated in a clinical trial for fluorescence-guided surgery of malignant brain tumors to assess its efficacy and tolerability.
  • The extent of tumor resection was verified by early postoperative magnetic resonance imaging.
  • RESULTS: Fluorescence staining of tumor tissue was bright in 11 patients (84%), resulting in complete resection of fluorescent tumor tissue in 9 patients (69%).
  • In 2 patients, residual fluorescent tumor tissue was also confirmed by magnetic resonance imaging.
  • Neither bleaching nor penetration of AFL-HSA into the surrounding brain edema or into necrotic tissue was seen.
  • The agreement between fluorescence and histopathology in tumor samples and samples of the tumor border was 83.3%.
  • CONCLUSION: Tumor fluorescence using AFL-HSA made fluorescence-guided brain tumor resection possible, demonstrating that albumin is a suitable carrier system for selective targeting of aminofluorescein into malignant gliomas.
  • [MeSH-major] Brain Neoplasms / pathology. Fluoresceins. Glioma / pathology. Serum Albumin
  • [MeSH-minor] Adult. Aged. Cell Proliferation. Female. Glioblastoma / pathology. Glioblastoma / surgery. Humans. Logistic Models. Magnetic Resonance Imaging. Male. Medulloblastoma / pathology. Medulloblastoma / surgery. Microscopy, Fluorescence. Middle Aged. Oligodendroglioma / pathology. Oligodendroglioma / surgery. Paraffin Embedding. Tissue Fixation

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  • (PMID = 19240573.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Fluoresceins; 0 / Serum Albumin; 3326-34-9 / 5-aminofluorescein
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32. De Sio L, Milano GM, Castellano A, Jenkner A, Fidani P, Dominici C, Donfrancesco A: Temozolomide in resistant or relapsed pediatric solid tumors. Pediatr Blood Cancer; 2006 Jul;47(1):30-6
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  • Tumor types were: neuroblastoma (NB; n = 17), medulloblastoma (MB; 8), brain stem glioma (BSG; 8), extraosseous Ewing's sarcoma/peripheral neuroectodermal tumor (EOES; 4), Ewing's sarcoma (ES; 4), anaplastic astrocytoma (AA; 3), rhabdomyosarcoma (RMS; 2), ependymoma (EP; 2), cerebral primitive neuroectodermal tumor (cPNET; 2), hepatocarcinoma (HC; 1), and osteosarcoma (OS; 1).
  • CONCLUSION: Oral TMZ was well tolerated in children with resistant or relapsed solid tumors and showed activity in NB and CNS tumours refractory to standard chemotherapy.
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Disease-Free Survival. Dose-Response Relationship, Drug. Female. Humans. Male. Survival Analysis

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  • [Copyright] Copyright 2006 Wiley-Liss, Inc.
  • [ErratumIn] Pediatr Blood Cancer. 2006 Oct 15;47(5):647-8
  • (PMID = 16047361.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Alkylating; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide
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33. Ueba T, Kadota E, Kano H, Yamashita K, Kageyama N: MATH-1 production by an adult medulloblastoma suggestive of a cerebellar external granule cell precursor origin. J Clin Neurosci; 2008 Jan;15(1):84-7
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  • [Title] MATH-1 production by an adult medulloblastoma suggestive of a cerebellar external granule cell precursor origin.
  • Radiological, histological and molecular findings in an uncommon adult case of cerebellar medulloblastoma suggested an external granular cell precursor origin.
  • Postoperative neuronal imaging studies showed that the tumor located in the cerebellar folia had been removed totally.
  • Pathological examination identified it as a desmoplastic medulloblastoma with subpial and subarachnoid infiltration and some infiltration into the molecular and granular layer via the perivascular space.
  • Polymerase chain reaction and immunohistochemical findings revealed the presence of MATH-1, expressed in cerebellar external granule cell precursors during fetal development, in the tumor cells.
  • These findings suggest that the tumor arose from external granule cell precursors of the cerebellum and that it was therefore of neuronal lineage.
  • [MeSH-major] Basic Helix-Loop-Helix Transcription Factors / metabolism. Cerebellar Neoplasms / metabolism. Cerebellar Neoplasms / pathology. Medulloblastoma / metabolism. Medulloblastoma / pathology. Neurons / physiology
  • [MeSH-minor] Adult. Female. Gene Expression Regulation, Neoplastic. Humans. Magnetic Resonance Imaging / methods. Neoplastic Stem Cells / physiology. Phosphopyruvate Hydratase / metabolism

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  • (PMID = 18032051.001).
  • [ISSN] 0967-5868
  • [Journal-full-title] Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
  • [ISO-abbreviation] J Clin Neurosci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Scotland
  • [Chemical-registry-number] 0 / ATOH1 protein, human; 0 / Basic Helix-Loop-Helix Transcription Factors; EC 4.2.1.11 / Phosphopyruvate Hydratase
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34. Bai R, Siu IM, Tyler BM, Staedtke V, Gallia GL, Riggins GJ: Evaluation of retinoic acid therapy for OTX2-positive medulloblastomas. Neuro Oncol; 2010 Jul;12(7):655-63
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  • [Title] Evaluation of retinoic acid therapy for OTX2-positive medulloblastomas.
  • The homeobox transcription factor OTX2 plays an essential role during embryonic brain development.
  • It is normally silenced in the adult brain, but is overexpressed by genomic amplification or other mechanisms in the majority of medulloblastomas (MBs).
  • These findings suggest a mechanism for RA-mediated anti-tumor effect on OTX2-positive MB cells and indicate that therapeutic targeting of OTX2 might be effective if FGF pathway-mediated resistance can be overcome.

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  • (PMID = 20511190.001).
  • [ISSN] 1523-5866
  • [Journal-full-title] Neuro-oncology
  • [ISO-abbreviation] Neuro-oncology
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / R01 NS052507
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Otx Transcription Factors; 0 / Otx2 protein, mouse; 5688UTC01R / Tretinoin
  • [Other-IDs] NLM/ PMC2940451
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35. Das P, Puri T, Suri V, Sharma MC, Sharma BS, Sarkar C: Medulloblastomas: a correlative study of MIB-1 proliferation index along with expression of c-Myc, ERBB2, and anti-apoptotic proteins along with histological typing and clinical outcome. Childs Nerv Syst; 2009 Jul;25(7):825-35
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  • [Title] Medulloblastomas: a correlative study of MIB-1 proliferation index along with expression of c-Myc, ERBB2, and anti-apoptotic proteins along with histological typing and clinical outcome.
  • BACKGROUND: Medulloblastoma (MB) is the most common pediatric brain tumor.
  • It is however rare in adults.
  • The genetic and protein expression profile of medulloblastoma is complex, which is worthwhile in terms of prognostication and development or selection of targeted therapy.
  • AIMS AND OBJECTIVES: The aims and objectives to correlate the MIB-1 proliferation index and protein expression profiles of c-Myc, ERBB2, and anti-apoptotic proteins (Bcl2 and Bcl-xL) in tumor cells with histological subtypes and clinical outcome.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / physiopathology. Cell Proliferation. Medulloblastoma / diagnosis. Medulloblastoma / physiopathology
  • [MeSH-minor] Adolescent. Adult. Brain / pathology. Brain / physiopathology. Child. Child, Preschool. Female. Follow-Up Studies. Humans. Ki-67 Antigen / metabolism. Male. Middle Aged. Prognosis. Proto-Oncogene Proteins c-bcl-2 / metabolism. Proto-Oncogene Proteins c-myc / metabolism. Receptor, ErbB-2 / metabolism. Young Adult. bcl-X Protein / metabolism

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  • (PMID = 19444455.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / BCL2L1 protein, human; 0 / Ki-67 Antigen; 0 / MYC protein, human; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Proto-Oncogene Proteins c-myc; 0 / bcl-X Protein; EC 2.7.10.1 / ERBB2 protein, human; EC 2.7.10.1 / Receptor, ErbB-2
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36. Ongürü O, Karslioglu Y, Ozcan A, Celik E: Anti-apoptotic and growth-promoting markers in adult medulloblastomas. Clin Neuropathol; 2010 Nov-Dec;29(6):384-9
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  • [Title] Anti-apoptotic and growth-promoting markers in adult medulloblastomas.
  • AIM: the aim of this study was to investigate the pathologic features, proliferation potential and expression of some anti-apoptotic and growth-promoting markers in adult medulloblastomas.
  • METHOD: we analyzed the immunohistochemical expression of survivin, c-KIT, Bcl-2, fascin, p-53 and Ki-67 in 18 adult medulloblastomas (> 16 years of age).
  • 14 cases were classical, 2 desmoplastic/nodular and 2 large cell medulloblastomas.
  • Moderate-to-high nuclear survivin expression was observed with high percentages (55 - 100%) in all medulloblastomas while Bcl-2 was mildly positive in only 1 case.
  • Fascin expression was observed in 13 medulloblastomas (72%), 9 of which showing moderate to high immunoreactivity.
  • CONCLUSION: frequent nuclear survivin expression implies the predominance of anti-apoptotic factors in pathogenesis of adult medulloblastomas.
  • It may also be a potential therapeutic target for adult medulloblastomas.
  • Although Blc-2 immunoreactivity was previously reported in approximately 30% in medulloblastomas, we have observed that it is rarely expressed in the present series of adult medulloblastomas.
  • To our knowledge, this is the first study evaluating fascin expression in medulloblastomas.
  • Mild-to-moderate cytoplasmic c-KIT immunoreactivity without membranous staining in adult medulloblastomas may support the previous studies reporting low level of c-KIT protein expression with lack of activating mutations in medulloblastomas.
  • It seems p53 is rarely involved in the course of develepment of adult medulloblastomas.
  • [MeSH-major] Apoptosis Regulatory Proteins / metabolism. Cerebellar Neoplasms / metabolism. Growth Substances / metabolism. Medulloblastoma / metabolism
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Carrier Proteins / metabolism. Female. Humans. Inhibitor of Apoptosis Proteins. Male. Microfilament Proteins / metabolism. Microtubule-Associated Proteins / metabolism. Middle Aged. Proto-Oncogene Proteins c-bcl-2 / metabolism. Proto-Oncogene Proteins c-kit / metabolism. Retrospective Studies. Tumor Suppressor Protein p53 / metabolism. Young Adult

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  • (PMID = 21073843.001).
  • [ISSN] 0722-5091
  • [Journal-full-title] Clinical neuropathology
  • [ISO-abbreviation] Clin. Neuropathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Apoptosis Regulatory Proteins; 0 / BIRC5 protein, human; 0 / Biomarkers, Tumor; 0 / Carrier Proteins; 0 / Growth Substances; 0 / Inhibitor of Apoptosis Proteins; 0 / Microfilament Proteins; 0 / Microtubule-Associated Proteins; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / Tumor Suppressor Protein p53; 146808-54-0 / fascin; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit
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37. Brandes AA, Franceschi E: Neuro-oncology: Genetic variation in pediatric and adult brain tumors. Nat Rev Neurol; 2010 Dec;6(12):653-4
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  • [Title] Neuro-oncology: Genetic variation in pediatric and adult brain tumors.
  • Two new studies suggest that pediatric medulloblastomas and high-grade gliomas are genetically different from the same tumors in adults.
  • Age-dependent gene expression might affect tumor biology; therefore, therapies for adult medulloblastomas or gliomas might not produce the same clinical outcomes in pediatric patients, and vice versa.
  • [MeSH-major] Brain Neoplasms / genetics. Cerebellar Neoplasms / genetics. Gene Expression Profiling. Glioma / genetics. Medulloblastoma / genetics
  • [MeSH-minor] Adult. Child. Genetic Variation. Humans

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  • (PMID = 21131914.001).
  • [ISSN] 1759-4766
  • [Journal-full-title] Nature reviews. Neurology
  • [ISO-abbreviation] Nat Rev Neurol
  • [Language] eng
  • [Publication-type] News
  • [Publication-country] England
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38. Nordfors K, Haapasalo J, Korja M, Niemelä A, Laine J, Parkkila AK, Pastorekova S, Pastorek J, Waheed A, Sly WS, Parkkila S, Haapasalo H: The tumour-associated carbonic anhydrases CA II, CA IX and CA XII in a group of medulloblastomas and supratentorial primitive neuroectodermal tumours: an association of CA IX with poor prognosis. BMC Cancer; 2010;10:148
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  • [Title] The tumour-associated carbonic anhydrases CA II, CA IX and CA XII in a group of medulloblastomas and supratentorial primitive neuroectodermal tumours: an association of CA IX with poor prognosis.
  • BACKGROUND: Medulloblastomas (MBs) and supratentorial primitive neuroectodermal tumours (PNETs) are the most common highly aggressive paediatric brain tumours.
  • [MeSH-major] Antigens, Neoplasm / analysis. Biomarkers, Tumor / analysis. Carbonic Anhydrase II / analysis. Carbonic Anhydrases / analysis. Cerebellar Neoplasms / enzymology. Medulloblastoma / enzymology. Neuroectodermal Tumors, Primitive / enzymology. Supratentorial Neoplasms / enzymology
  • [MeSH-minor] Adolescent. Adult. Aged. Apoptosis. Chi-Square Distribution. Child. Child, Preschool. Cytoplasm / enzymology. Endothelial Cells / enzymology. Female. Finland. Humans. Immunohistochemistry. Infant. Infant, Newborn. Kaplan-Meier Estimate. Male. Middle Aged. Odds Ratio. Proportional Hazards Models. Time Factors. Treatment Outcome. Young Adult

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  • (PMID = 20398423.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; EC 4.2.1.- / Carbonic Anhydrase II; EC 4.2.1.1 / CA9 protein, human; EC 4.2.1.1 / Carbonic Anhydrases; EC 4.2.1.1 / carbonic anhydrase XII
  • [Other-IDs] NLM/ PMC2874782
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39. Yamashita Y, Kumabe T, Higano S, Watanabe M, Tominaga T: Minimum apparent diffusion coefficient is significantly correlated with cellularity in medulloblastomas. Neurol Res; 2009 Nov;31(9):940-6
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  • [Title] Minimum apparent diffusion coefficient is significantly correlated with cellularity in medulloblastomas.
  • The purpose of this study was to evaluate the relationship between ADC and tumor cellularity in medulloblastoma and other posterior fossa tumors.
  • METHODS: Pre-operative diffusion-weighted MR images were retrospectively reviewed in 26 patients with posterior fossa neoplasms: 11 medulloblastomas, one atypical teratoid/rhabdoid tumor (AT/RT), four glioblastomas, four ependymomas, three pilocytic astrocytomas and three hemangioblastomas.
  • The minimum ADC (minADC) value of each tumor was determined on ADC maps derived from isotropic diffusion-weighted MR images.
  • Tumor cellularity measured in surgical specimens was compared with the minADC value by simple linear regression analysis.
  • RESULTS: The mean minADC value of the medulloblastoma was significantly lower than those of ependymoma, pilocytic astrocytoma and hemangioblastoma without overlap in the range of minADC values.
  • Therefore, medulloblastomas could be clearly differentiated by absolute minADC values.
  • AT/RT and glioblastoma had similar minADC values to medulloblastoma.
  • Tumor cellularity was negatively correlated with the minADC value in medulloblastomas and other posterior fossa tumors.
  • DISCUSSION: The low minADC value of medulloblastomas reflects the high tumor cellularity.
  • Analysis of ADC values has high predictive value for the differentiation of medulloblastoma from other posterior fossa tumors.
  • [MeSH-major] Brain Neoplasms / pathology. Diffusion Magnetic Resonance Imaging / methods. Medulloblastoma / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Astrocytoma / metabolism. Astrocytoma / pathology. Astrocytoma / physiopathology. Body Water / physiology. Cell Count. Child. Child, Preschool. Diagnosis, Differential. Diffusion. Ependymoma / metabolism. Ependymoma / pathology. Ependymoma / physiopathology. Extracellular Space / metabolism. Female. Hemangioblastoma / metabolism. Hemangioblastoma / pathology. Hemangioblastoma / physiopathology. Humans. Infant. Male. Middle Aged. Retrospective Studies. Young Adult

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  • (PMID = 19138469.001).
  • [ISSN] 1743-1328
  • [Journal-full-title] Neurological research
  • [ISO-abbreviation] Neurol. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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40. Li XN, Shu Q, Su JM, Adesina AM, Wong KK, Perlaky L, Antalffy BA, Blaney SM, Lau CC: Differential expression of survivin splice isoforms in medulloblastomas. Neuropathol Appl Neurobiol; 2007 Feb;33(1):67-76
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  • [Title] Differential expression of survivin splice isoforms in medulloblastomas.
  • The current study was undertaken to examine the mRNA expression of survivin isoforms and their correlation with clinical staging and outcome in 20 medulloblastoma (MB) tumours, three MB cell lines and normal brain tissues (a foetal and an adult cerebellum) by densitometry scanning of 32p-dCTP incorporated reverse transcription polymerase chain reaction (RT-PCR) products and quantitative real-time PCR.
  • Our results showed that the normal adult brain only expressed low levels of survivin-deltaEx3 mRNA, while the foetal brain expressed all three isoforms, with wild-type survivin as the dominant transcript.
  • [MeSH-major] Cerebellar Neoplasms / metabolism. Medulloblastoma / metabolism. Microtubule-Associated Proteins / biosynthesis. Neoplasm Proteins / biosynthesis
  • [MeSH-minor] Adolescent. Cell Line, Tumor. Child. Child, Preschool. Female. Gene Expression Regulation, Neoplastic. Glyceraldehyde-3-Phosphate Dehydrogenases / metabolism. Humans. Immunohistochemistry. Infant. Inhibitor of Apoptosis Proteins. Isomerism. Male. Neoplasm Staging. RNA, Messenger / biosynthesis. Reverse Transcriptase Polymerase Chain Reaction. Treatment Outcome

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  • (PMID = 17239009.001).
  • [ISSN] 0305-1846
  • [Journal-full-title] Neuropathology and applied neurobiology
  • [ISO-abbreviation] Neuropathol. Appl. Neurobiol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins; 0 / RNA, Messenger; EC 1.2.1.- / Glyceraldehyde-3-Phosphate Dehydrogenases
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41. Yang LS, Wang YQ, Huang FP: [Correlation between the prognosis of medulloblastoma and relevant clinical factors: analysis of 73 cases]. Zhonghua Yi Xue Za Zhi; 2007 May 22;87(19):1322-5
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  • [Title] [Correlation between the prognosis of medulloblastoma and relevant clinical factors: analysis of 73 cases].
  • OBJECTIVE: To analyze the correlation between the prognosis of medulloblastoma (MB) and relevant clinical factors.
  • The correlation between the prognosis and the clinical factors, such and sex, age, tumor location, extent of tumor resection, brainstem invasion, radiotherapy, chemotherapy, ventriculoperitoneal shunt and glial differentiation was analyzed.
  • Those undergoing whole brain/posterior fossa plus spinal axis radiotherapy showed a better prognosis than those undergoing whole brain/posterior fossa radiotherapy.
  • [MeSH-major] Central Nervous System Neoplasms / therapy. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Kaplan-Meier Estimate. Male. Prognosis. Retrospective Studies. Survival Rate

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  • (PMID = 17727776.001).
  • [ISSN] 0376-2491
  • [Journal-full-title] Zhonghua yi xue za zhi
  • [ISO-abbreviation] Zhonghua Yi Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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42. Viana-Pereira M, Almeida I, Sousa S, Mahler-Araújo B, Seruca R, Pimentel J, Reis RM: Analysis of microsatellite instability in medulloblastoma. Neuro Oncol; 2009 Oct;11(5):458-67
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  • [Title] Analysis of microsatellite instability in medulloblastoma.
  • Medulloblastoma is the most common malignant brain tumor in children.
  • The presence of microsatellite instability (MSI) in brain tumors, particularly medulloblastomas, has not been properly addressed.
  • The aim of the present study was to evaluate the role of MSI in medulloblastoma carcinogenesis.
  • This study is the most comprehensive analysis of MSI in medulloblastomas to date.
  • We observed the presence of MSI together with mutations of MSI target genes in a small fraction of cases, suggesting a new genetic pathway for a role in medulloblastoma development.
  • [MeSH-major] Biomarkers, Tumor / genetics. Cerebellar Neoplasms / genetics. Medulloblastoma / genetics. Microsatellite Instability
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. DNA Methylation. DNA Mutational Analysis. Female. Humans. Immunohistochemistry. Infant. Male. Middle Aged. Polymerase Chain Reaction. Polymorphism, Single-Stranded Conformational. Young Adult


43. Gonçalves MI, Radzinsky TC, da Silva NS, Chiari BM, Consonni D: Speech-language and hearing complaints of children and adolescents with brain tumors. Pediatr Blood Cancer; 2008 Mar;50(3):706-8
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  • [Title] Speech-language and hearing complaints of children and adolescents with brain tumors.
  • Central nervous system (CNS) tumors generally leave sequelae that may compromise speech, language, swallowing, hearing, and voice functions.
  • This report describes the incidence of speech-language and hearing complaints and disorders in children and adolescents with CNS tumor under treatment at one of the most important Brazilian reference center for pediatric cancer.
  • [MeSH-major] Brain Neoplasms / complications. Hearing Loss / etiology. Language Disorders / etiology. Speech Disorders / etiology
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / adverse effects. Antineoplastic Agents / therapeutic use. Astrocytoma / complications. Astrocytoma / drug therapy. Child. Child, Preschool. Craniopharyngioma / complications. Craniopharyngioma / drug therapy. Deglutition Disorders / etiology. Ependymoma / complications. Ependymoma / drug therapy. Facial Paralysis / etiology. Female. Humans. Infant. Male. Medulloblastoma / complications. Medulloblastoma / drug therapy


44. Oba-Shinjo SM, Caballero OL, Jungbluth AA, Rosemberg S, Old LJ, Simpson AJ, Marie SK: Cancer-testis (CT) antigen expression in medulloblastoma. Cancer Immun; 2008;8:7
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  • [Title] Cancer-testis (CT) antigen expression in medulloblastoma.
  • Medulloblastoma is the most common childhood malignant tumor of the central nervous system.
  • Treatment of medulloblastoma requires harmful therapy and nevertheless carries a poor prognosis.
  • Due to their presence in various cancers and their limited expression in normal tissues, CT antigens are ideal vaccine targets for tumor immunotherapy.
  • CT antigens, such as MAGE and NY-ESO-1, have been employed in clinical trials in various malignancies but little is known about their presence in medulloblastoma.
  • We analyzed 25 medulloblastomas for the expression of a panel of CT antigens by RT-PCR and immunohistochemistry.
  • The absence of correlation between mRNA and protein expression in medulloblastoma has not been observed in other tumors and further studies addressing the biology of CT antigens are necessary to investigate the present discrepant results.
  • [MeSH-major] Antigens, Neoplasm / biosynthesis. Cerebellar Neoplasms / immunology. Medulloblastoma / immunology
  • [MeSH-minor] Adult. Cancer Vaccines. Child. Female. Gene Expression Regulation, Neoplastic. Humans. Male. Membrane Proteins / biosynthesis. Membrane Proteins / genetics. Neoplasm Proteins / biosynthesis. Neoplasm Proteins / genetics. RNA Processing, Post-Transcriptional / genetics. RNA, Messenger / metabolism. Testis / metabolism. Testis / pathology

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  • (PMID = 18426187.001).
  • [ISSN] 1424-9634
  • [Journal-full-title] Cancer immunity
  • [ISO-abbreviation] Cancer Immun.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / CTAG1B protein, human; 0 / Cancer Vaccines; 0 / MAGEA3 protein, human; 0 / MAGEC1 protein, human; 0 / MAGEC2 protein, human; 0 / Membrane Proteins; 0 / Neoplasm Proteins; 0 / RNA, Messenger
  • [Other-IDs] NLM/ PMC2935780
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45. Lindsey JC, Lusher ME, Anderton JA, Gilbertson RJ, Ellison DW, Clifford SC: Epigenetic deregulation of multiple S100 gene family members by differential hypomethylation and hypermethylation events in medulloblastoma. Br J Cancer; 2007 Jul 16;97(2):267-74
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  • [Title] Epigenetic deregulation of multiple S100 gene family members by differential hypomethylation and hypermethylation events in medulloblastoma.
  • Using a pharmacological expression reactivation approach, we screened 16 S100 genes for evidence of epigenetic regulation in medulloblastoma, the most common malignant brain tumour of childhood.
  • Four family members (S100A2, S100A4, S100A6 and S100A10) demonstrated evidence of upregulated expression in multiple medulloblastoma cell lines, following treatment with the DNA methyltransferase inhibitor, 5'-aza-2'-deoxycytidine.
  • Assessment of these genes in the non-neoplastic cerebellum (from which medulloblastomas develop) revealed strong somatic methylation affecting S100A2 and S100A4, whereas S100A6 and S100A10 were unmethylated.
  • Assessed against these normal tissue-specific methylation states, S100A6 and S100A10 demonstrated tumour-specific hypermethylation in medulloblastoma primary tumours (5 out of 40 and 4 out of 35, respectively, both 12%) and cell lines (both 7 out of 9, 78%), which was associated with their transcriptional silencing.
  • In summary, these data characterise complex patterns of somatic methylation affecting S100 genes in the normal cerebellum and demonstrate their disruption causing epigenetic deregulation of multiple S100 family members in medulloblastoma development.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Epigenesis, Genetic. Gene Expression Regulation, Neoplastic. Medulloblastoma / genetics. S100 Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Azacitidine / analogs & derivatives. Azacitidine / pharmacology. Cell Line, Tumor. Cerebellum / metabolism. Child. Child, Preschool. DNA Methylation. DNA Modification Methylases / antagonists & inhibitors. Female. Humans. Infant. Male

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  • (PMID = 17579622.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / S100 Proteins; 776B62CQ27 / decitabine; EC 2.1.1.- / DNA Modification Methylases; M801H13NRU / Azacitidine
  • [Other-IDs] NLM/ PMC2360310
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46. Onilude OE, Lusher ME, Lindsey JC, Pearson AD, Ellison DW, Clifford SC: APC and CTNNB1 mutations are rare in sporadic ependymomas. Cancer Genet Cytogenet; 2006 Jul 15;168(2):158-61
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  • The ependymoma is the second most common malignant brain tumor of childhood; however, its molecular basis is poorly understood.
  • The formation of multiple ependymomas has been reported as an occasional feature of Turcot syndrome type 2 (TS2), a familial cancer syndrome caused by inherited mutations of the APC tumor suppressor gene, and characterised by the concurrence of a primary CNS tumor (predominantly medulloblastoma) and multiple colorectal adenomas.
  • APC is a critical component of the Wnt/Wingless signaling pathway, which is disrupted in sporadic cancers (e.g., colorectal adenomas, hepatocellular carcinomas, and medulloblastomas) by somatic mutations affecting multiple genes encoding alternative pathway components, including APC and CTNNB1 (encoding beta-catenin).
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Chromatography, High Pressure Liquid. DNA Mutational Analysis. Female. Humans. Male. Middle Aged

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  • (PMID = 16843107.001).
  • [ISSN] 0165-4608
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adenomatous Polyposis Coli Protein; 0 / CTNNB1 protein, human; 0 / beta Catenin
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47. De Bortoli M, Castellino RC, Lu XY, Deyo J, Sturla LM, Adesina AM, Perlaky L, Pomeroy SL, Lau CC, Man TK, Rao PH, Kim JY: Medulloblastoma outcome is adversely associated with overexpression of EEF1D, RPL30, and RPS20 on the long arm of chromosome 8. BMC Cancer; 2006 Sep 12;6:223
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  • [Title] Medulloblastoma outcome is adversely associated with overexpression of EEF1D, RPL30, and RPS20 on the long arm of chromosome 8.
  • BACKGROUND: Medulloblastoma is the most common malignant brain tumor of childhood.
  • In the present study, we applied cytogenetic characterization to guide the identification of biologically significant genes from gene expression microarray profiles of medulloblastoma.
  • METHODS: We analyzed 71 primary medulloblastomas for chromosomal copy number aberrations (CNAs) using comparative genomic hybridization (CGH).
  • By applying CGH results to gene expression analysis of medulloblastoma, we identified three 8q-mapped genes that are associated with overall survival in the larger group of 64 patients (p < 0.05): eukaryotic translation elongation factor 1D (EEF1D), ribosomal protein L30 (RPL30), and ribosomal protein S20 (RPS20).
  • CONCLUSION: The complementary use of CGH and expression profiles can facilitate the identification of clinically significant candidate genes involved in medulloblastoma growth.
  • We demonstrate that gain of 8q and expression levels of three 8q-mapped candidate genes (EEF1D, RPL30, RPS20) are associated with adverse outcome in medulloblastoma.

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  • (PMID = 16968546.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA105607; United States / NICHD NIH HHS / HD / HD042977; United States / NINDS NIH HHS / NS / NS043517; United States / NICHD NIH HHS / HD / T32 HD042977; United States / NCI NIH HHS / CA / R01 CA105607; United States / NINDS NIH HHS / NS / K08 NS043517; United States / NICHD NIH HHS / HD / K12 HD041648; United States / NCI NIH HHS / CA / R01 CA109467; United States / NICHD NIH HHS / HD / HD041648; United States / NCI NIH HHS / CA / CA109467
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Peptide Elongation Factor 1; 0 / Ribosomal Proteins; 0 / ribosomal protein L30; 0 / ribosomal protein S20
  • [Other-IDs] NLM/ PMC1578584
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48. Lueth M, von Deimling A, Pietsch T, Wong LJ, Kurtz A, Henze G, Driever PH: Medulloblastoma harbor somatic mitochondrial DNA mutations in the D-loop region. J Pediatr Hematol Oncol; 2010 Mar;32(2):156-9
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  • [Title] Medulloblastoma harbor somatic mitochondrial DNA mutations in the D-loop region.
  • Despite the growing knowledge on molecular risk factors of the most common malignant brain tumor in childhood, medulloblastoma, its biology remains only partially understood.
  • A previous study investigating the entire mitochondrial genome of medulloblastoma revealed a number of somatic mutations in tumor and corresponding cerebrospinal fluid samples.
  • In our present study we sought to corroborate these results on somatic and germ line mutations by comparing the complete mitochondrial genome sequences of medulloblastoma tissue in a further cohort of patients.
  • Analysis of the entire mitochondrial genome by temporal temperature gel electrophoresis and direct sequencing revealed 6 somatic mutations in 6 of 15 medulloblastoma.
  • These results are in support of our previous findings on frequency of somatic mitochondrial mutations in medulloblastoma.
  • [MeSH-major] Cerebellar Neoplasms / genetics. DNA, Mitochondrial / genetics. Medulloblastoma / genetics. Mutation
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Genome, Mitochondrial. Humans. Infant. Male

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  • (PMID = 20147852.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Mitochondrial
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49. Jacobs AH, Thomas A, Kracht LW, Li H, Dittmar C, Garlip G, Galldiks N, Klein JC, Sobesky J, Hilker R, Vollmar S, Herholz K, Wienhard K, Heiss WD: 18F-fluoro-L-thymidine and 11C-methylmethionine as markers of increased transport and proliferation in brain tumors. J Nucl Med; 2005 Dec;46(12):1948-58
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  • [Title] 18F-fluoro-L-thymidine and 11C-methylmethionine as markers of increased transport and proliferation in brain tumors.
  • Because of the high glucose metabolism in normal brain tissue 18F-FDG is not the ideal tracer for the detection of gliomas.
  • Methyl-11C-l-methionine (11C-MET) is better suited for imaging the extent of gliomas, because it is transported specifically into tumors but only insignificantly into normal brain.
  • 3'-Deoxy-3'-18F-fluorothymidine (18F-FLT) has been introduced as a proliferation marker in a variety of neoplasias and has promising potential for the detection of brain tumors, because its uptake in normal brain is low.
  • Kinetic modeling was performed on 14 patients for regional time-activity curves of 18F-FLT from tumorous and normal brain tissue.
  • Tumor volumes detected by 18F-FLT and 11C-MET were larger than tumor regions displaying gadolinium enhancement (P<0.01).
  • Some tumor regions were detected only by either 18F-FLT (7 patients) or 11C-MET (13 patients).
  • Kinetic modeling revealed that 18F-FLT uptake in tumor tissue seems to be predominantly due to elevated transport and net influx.
  • CONCLUSION: 18F-FLT is a promising tracer for the detection and characterization of primary central nervous system tumors and might help to differentiate between low- and high-grade gliomas.
  • In some tumors and tumor areas, 18F-FLT uptake is not related to 11C-MET uptake.
  • [MeSH-major] Antiviral Agents / pharmacology. Astrocytoma / radionuclide imaging. Brain Neoplasms / radionuclide imaging. Dideoxynucleosides / pharmacology. Glioma / radionuclide imaging. Medulloblastoma / radionuclide imaging. Methionine / analogs & derivatives. Radiopharmaceuticals / pharmacology
  • [MeSH-minor] Adult. Aged. Biological Transport. Cell Proliferation. False Positive Reactions. Female. Humans. Kinetics. Magnetic Resonance Imaging / methods. Male. Middle Aged. Phosphorylation. Positron-Emission Tomography. Sensitivity and Specificity. Time Factors

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  • (PMID = 16330557.001).
  • [ISSN] 0161-5505
  • [Journal-full-title] Journal of nuclear medicine : official publication, Society of Nuclear Medicine
  • [ISO-abbreviation] J. Nucl. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antiviral Agents; 0 / Dideoxynucleosides; 0 / Radiopharmaceuticals; AE28F7PNPL / Methionine; BN630929UL / methionine methyl ester; PG53R0DWDQ / alovudine
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50. Nicholson HS, Kretschmar CS, Krailo M, Bernstein M, Kadota R, Fort D, Friedman H, Harris MB, Tedeschi-Blok N, Mazewski C, Sato J, Reaman GH: Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children's Oncology Group. Cancer; 2007 Oct 1;110(7):1542-50
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  • [Title] Phase 2 study of temozolomide in children and adolescents with recurrent central nervous system tumors: a report from the Children's Oncology Group.
  • BACKGROUND: Effective chemotherapy is lacking for most types of central nervous system (CNS) tumors in children.
  • Temozolomide, an agent with activity against adult brain tumors, was investigated in children and adolescents with recurrent CNS tumors.
  • RESULTS: The cohort comprised 122 patients, including 113 with CNS tumors.
  • Among 104 evaluable patients with CNS tumors, 5 PRs and 1 CR were observed.
  • PRs occurred in 1 of 23 evaluable patients with high-grade astrocytoma, 1 of 21 with low-grade astrocytoma, and 3 of 25 with medulloblastoma/primitive neuroectodermal tumor (PNET).
  • The CR occurred in an additional patient with medulloblastoma/PNET.
  • No responses were observed in patients with ependymoma, brain-stem glioma, or other CNS tumors.
  • CONCLUSIONS: Although overall objective responses were limited, further exploration of temozolomide may be warranted in children with medulloblastoma and other PNETs, or in patients with low-grade astrocytoma, perhaps in a setting of less pretreatment than the patients in the current study, or in the context of multiagent therapy.
  • [MeSH-major] Antineoplastic Agents, Alkylating / therapeutic use. Brain Neoplasms / drug therapy. Dacarbazine / analogs & derivatives. Neoplasm Recurrence, Local / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Astrocytoma / drug therapy. Central Nervous System Neoplasms / drug therapy. Child. Child, Preschool. Drug Administration Schedule. Ependymoma / drug therapy. Female. Humans. Infant. Male. Medulloblastoma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Treatment Outcome


51. Hambardzumyan D, Becher OJ, Holland EC: Cancer stem cells and survival pathways. Cell Cycle; 2008 May 15;7(10):1371-8
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  • Gliomas and medulloblastomas are the most frequent malignant brain tumors in adult and children respectively.
  • Although both tumors arise in the CNS, there is a significant difference in their therapeutic response.
  • Medulloblastomas are relatively curable, while glioblastomas are basically incurable.
  • During the last decade several reports have demonstrated the existence of cancer stem cells in brain tumors, their location and their response to treatment.
  • We have recently described the therapeutic response of medulloblastomas to radiation in their native microenvironment, illustrating how p53 and Pi3K signaling pathways lead to the evasion of cell death by the nestin-expressing cells in the perivascular stem cell niche, even while the bulk of tumor succumbs to apoptosis.(1) It remains to be determined whether this mechanism of tumor resistance applies to the more complex stem-cell niche and tumor bulk of gliomas.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Medulloblastoma / radiotherapy. Neoplastic Stem Cells / radiation effects. Signal Transduction / radiation effects
  • [MeSH-minor] Animals. Humans. Intermediate Filament Proteins / metabolism. Mice. Models, Biological. Nerve Tissue Proteins / metabolism. Nestin. Phosphatidylinositol 3-Kinases / metabolism. Receptors, Notch / metabolism. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 18421251.001).
  • [ISSN] 1551-4005
  • [Journal-full-title] Cell cycle (Georgetown, Tex.)
  • [ISO-abbreviation] Cell Cycle
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01 5R01CA100688-2; United States / NCI NIH HHS / CA / R01CA 5R01CA099489-1
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Intermediate Filament Proteins; 0 / NES protein, human; 0 / Nerve Tissue Proteins; 0 / Nes protein, mouse; 0 / Nestin; 0 / Receptors, Notch; 0 / Tumor Suppressor Protein p53; EC 2.7.1.- / Phosphatidylinositol 3-Kinases
  • [Number-of-references] 112
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52. Hu WW, Zheng XJ, Shen G, Liu WG, Shen H, Fu WM, Zhou JY: [Diagnosis and micro-neurosurgery for the fourth cerebral ventricle tumors]. Zhonghua Zhong Liu Za Zhi; 2007 Feb;29(2):144-6
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  • METHODS: Tumor of the fourth ventricle was clinically diagnosed in 86 patients basing on the preliminary assessment of symptom and CT or MRI findings.
  • The pathology included 32 medulloblastomas, 23 ependymoma, 15 astrocytoma, 10 hemangiblastomas, 2 choroid plexus papillomas, and 4 epidermoid cysts.
  • CONCLUSION: Medulloblastoma, astrocytoma and hemangiblastoma are suggested to be removed totally whenever technically possible according to the site, character and volume of the tumor.
  • For ependymoma, if close to the brain stem, is recommended to be subtotally removed.
  • [MeSH-major] Cerebral Ventricle Neoplasms / diagnosis. Fourth Ventricle / pathology. Medulloblastoma / diagnosis. Microsurgery / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Astrocytoma / diagnosis. Astrocytoma / radiography. Astrocytoma / surgery. Child. Child, Preschool. Combined Modality Therapy. Ependymoma / diagnosis. Ependymoma / radiography. Ependymoma / surgery. Female. Follow-Up Studies. Hemangioblastoma / diagnosis. Hemangioblastoma / radiography. Hemangioblastoma / surgery. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Recurrence, Local. Survival Analysis. Survival Rate. Tomography, X-Ray Computed

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  • (PMID = 17645855.001).
  • [ISSN] 0253-3766
  • [Journal-full-title] Zhonghua zhong liu za zhi [Chinese journal of oncology]
  • [ISO-abbreviation] Zhonghua Zhong Liu Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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53. Inda MM, Castresana JS: RASSF1A promoter is highly methylated in primitive neuroectodermal tumors of the central nervous system. Neuropathology; 2007 Aug;27(4):341-6
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  • [Title] RASSF1A promoter is highly methylated in primitive neuroectodermal tumors of the central nervous system.
  • Although cancer is rare in children, primary brain tumors constitute the most frequent location of solid tumors in childhood.
  • Primitive neuroectodermal tumors (PNET) of the central nervous system can be divided into infratentorial PNET or medulloblastoma (MB), and supratentorial (sPNET) tumors.
  • The RASSF1A (Ras Association Domain Family Protein 1) gene, located at 3p21.3, is highly methylated in multiple primary tumor samples, including neuroblastoma.
  • Therefore, the inactivation of the RASSF1A gene seems to be an important step in the tumorigenesis of PNET of the central nervous sytem.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Medulloblastoma / genetics. Neuroectodermal Tumors, Primitive / genetics. Promoter Regions, Genetic. Supratentorial Neoplasms / genetics. Tumor Suppressor Proteins / genetics
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. DNA Methylation. Female. Humans. Male. Middle Aged. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 17899687.001).
  • [ISSN] 0919-6544
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / RASSF1 protein, human; 0 / Tumor Suppressor Proteins
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54. Rieken S, Gaiser T, Mohr A, Welzel T, Witt O, Kulozik AE, Wick W, Debus J, Combs SE: Outcome and prognostic factors of desmoplastic medulloblastoma treated within a multidisciplinary treatment concept. BMC Cancer; 2010;10:450
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  • [Title] Outcome and prognostic factors of desmoplastic medulloblastoma treated within a multidisciplinary treatment concept.
  • BACKGROUND: Desmoplasia in medulloblastoma is often diagnosed in adult patients and was repeatedly associated with improved results.
  • Today, all medulloblastoma patients receive intensive multimodal treatment including surgery, radiotherapy and chemotherapy.
  • This study was set up to investigate treatment outcome and prognostic factors after radiation therapy in patients with desmoplastic medulloblastomas.
  • METHODS: Twenty patients treated for desmoplastic medulloblastoma in the Department of Radiation Oncology at the University of Heidelberg between 1984 and 2007 were included.
  • Tumor resection was performed in all patients.
  • Two patients underwent whole brain radiotherapy (WBRT), and 18 patients received craniospinal irradiation (CSI).
  • The median dose to the whole brain and the craniospinal axis was 35.2 Gray (Gy), and 54.4 Gy to the posterior fossa.
  • Five patients died from recurrent medulloblastoma.
  • CONCLUSIONS: Multimodal approaches with surgical resection followed by chemoirradiation achieved high response rates with long OS in desmoplastic medulloblastoma patients.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cerebellar Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy. Neoplasm Recurrence, Local / therapy. Neurosurgical Procedures
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoplasm Staging. Radiotherapy Dosage. Retrospective Studies. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 20731859.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2939548
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55. Wells EM, Walsh KS, Khademian ZP, Keating RF, Packer RJ: The cerebellar mutism syndrome and its relation to cerebellar cognitive function and the cerebellar cognitive affective disorder. Dev Disabil Res Rev; 2008;14(3):221-8
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  • The postoperative cerebellar mutism syndrome (CMS), consisting of diminished speech output, hypotonia, ataxia, and emotional lability, occurs after surgery in up to 25% of patients with medulloblastoma and occasionally after removal of other posterior fossa tumors.
  • CMS shares many similarities with the cerebellar cognitive affective syndrome, more commonly described in adults and consisting of disturbances of executive function, visuospatial skills, nonmotor language, and affect regulation.
  • [MeSH-major] Brain Damage, Chronic / etiology. Cerebellar Neoplasms / surgery. Cognition Disorders / etiology. Cranial Fossa, Posterior / surgery. Developmental Disabilities / etiology. Medulloblastoma / surgery. Mood Disorders / etiology. Mutism / etiology. Skull Base Neoplasms / surgery. Survivors / psychology
  • [MeSH-minor] Adolescent. Adult. Child. Child Behavior Disorders / diagnosis. Child Behavior Disorders / etiology. Child Behavior Disorders / psychology. Follow-Up Studies. Humans. Risk Factors. Young Adult


56. Kadota RP, Mahoney DH, Doyle J, Duerst R, Friedman H, Holmes E, Kun L, Zhou T, Pollack IF: Dose intensive melphalan and cyclophosphamide with autologous hematopoietic stem cells for recurrent medulloblastoma or germinoma. Pediatr Blood Cancer; 2008 Nov;51(5):675-8
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  • [Title] Dose intensive melphalan and cyclophosphamide with autologous hematopoietic stem cells for recurrent medulloblastoma or germinoma.
  • PURPOSE: To determine the response, toxicity, and survival for children with progressive or recurrent medulloblastoma and germinoma using a single myeloablative course of chemotherapy supported by autologous hematopoietic stem cells.
  • There were 6 medulloblastoma and 3 germinoma survivors with a median follow-up of 7.5 years (range = 2.8-10).
  • CONCLUSION: Myeloablative chemotherapy consisting of cyclophosphamide and melphalan was tolerable in the relapsed brain tumor setting with 19/29 cases achieving CR or CCR status and 9/29 becoming long-term survivors.

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
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  • (PMID = 18623206.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA098543-06; United States / NCI NIH HHS / CA / CA98543; United States / NCI NIH HHS / CA / U10 CA098413-06; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA098543; None / None / / U10 CA098413-06; None / None / / U10 CA098543-06
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 8N3DW7272P / Cyclophosphamide; Q41OR9510P / Melphalan
  • [Other-IDs] NLM/ NIHMS123029; NLM/ PMC2900925
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57. Deen HG, Miller DA, Kostick DA, Jaeckle KA: Removal of an orbital metallic foreign body to facilitate magnetic resonance imaging: technical case report. Neurosurgery; 2006 May;58(5):E999; discussion E999
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  • OBJECTIVE AND IMPORTANCE: Magnetic resonance imaging (MRI) is the imaging modality of choice for brain tumors and other lesions of the central nervous system.
  • CLINICAL PRESENTATION: Two patients, one with a posterior fossa mass and one with suspected central nervous system lymphoma, were seen at our institution.
  • The first patient underwent posterior fossa craniotomy and removal of the tumor, which proved to be a medulloblastoma.
  • CONCLUSION: Two patients with central nervous system tumors underwent removal of a metal fragment in the orbit for the specific purpose of facilitating MRI scans.
  • [MeSH-minor] Adult. Cerebellar Neoplasms / diagnosis. Humans. Male. Medulloblastoma / diagnosis. Meningeal Neoplasms / diagnosis. Middle Aged. Orbit / radiography. Orbit / surgery

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  • (PMID = 16639311.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Ferric Compounds; 1317-54-0 / ferrite
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58. Gururangan S, Krauser J, Watral MA, Driscoll T, Larrier N, Reardon DA, Rich JN, Quinn JA, Vredenburgh JJ, Desjardins A, McLendon RE, Fuchs H, Kurtzberg J, Friedman HS: Efficacy of high-dose chemotherapy or standard salvage therapy in patients with recurrent medulloblastoma. Neuro Oncol; 2008 Oct;10(5):745-51
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  • [Title] Efficacy of high-dose chemotherapy or standard salvage therapy in patients with recurrent medulloblastoma.
  • The efficacy of high-dose chemotherapy (HDC) or standard salvage therapy was evaluated in patients with recurrent medulloblastoma (MBL) using retrospective chart review of all patients with recurrent MBL treated at Duke University Medical Center between 1995 and 2005 and who had undergone HDC with or without radiotherapy (RT) or standard salvage therapy after relapse.
  • All patients in groups B and C have died of tumor, at a median of 35 months and 26 months from HDC and standard salvage therapy, respectively.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Cerebellar Neoplasms / drug therapy. Medulloblastoma / drug therapy. Neoplasm Recurrence, Local / drug therapy. Salvage Therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Disease-Free Survival. Humans. Kaplan-Meier Estimate. Retrospective Studies


59. Howes TL, Buatti JM, Kirby PA, Carlisle TL, Ryken TC: Radiation induced adult medulloblastoma: a case report. J Neurooncol; 2006 Nov;80(2):191-4
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  • [Title] Radiation induced adult medulloblastoma: a case report.
  • Adult medulloblastoma is a rare intracranial tumor.
  • Our patient is a 61 year old woman treated with cranial irradiation 15 years previously for a low grade astrocytoma in the left posterior temporal lobe that was recently diagnosed with medulloblastoma in the right cerebellum.
  • This is the first reported case of radiation induced adult medulloblastoma.
  • [MeSH-major] Cerebellar Neoplasms / etiology. Medulloblastoma / etiology. Neoplasms, Radiation-Induced / pathology
  • [MeSH-minor] Astrocytoma / radiotherapy. Brain Neoplasms / radiotherapy. Craniotomy. Female. Humans. Magnetic Resonance Imaging. Middle Aged. Neurosurgical Procedures. Temporal Lobe / pathology

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  • (PMID = 16710747.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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60. Kieran MW, Packer RJ, Onar A, Blaney SM, Phillips P, Pollack IF, Geyer JR, Gururangan S, Banerjee A, Goldman S, Turner CD, Belasco JB, Broniscer A, Zhu Y, Frank E, Kirschmeier P, Statkevich P, Yver A, Boyett JM, Kun LE: Phase I and pharmacokinetic study of the oral farnesyltransferase inhibitor lonafarnib administered twice daily to pediatric patients with advanced central nervous system tumors using a modified continuous reassessment method: a Pediatric Brain Tumor Consortium Study. J Clin Oncol; 2007 Jul 20;25(21):3137-43
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  • [Title] Phase I and pharmacokinetic study of the oral farnesyltransferase inhibitor lonafarnib administered twice daily to pediatric patients with advanced central nervous system tumors using a modified continuous reassessment method: a Pediatric Brain Tumor Consortium Study.
  • PURPOSE: A dose-escalation phase I and pharmacokinetic study of the farnesyltransferase inhibitor lonafarnib (SCH66336) was conducted in children with recurrent or progressive CNS tumors.
  • RESULTS: Fifty-three children with progressive or recurrent brain tumors were enrolled, with a median age of 12.2 years (range, 3.9 to 19.5 years).
  • Both radiographic response (one anaplastic astrocytoma) and stable disease (one medulloblastoma, two high-grade and four low-grade gliomas, one ependymoma, and one sarcoma) were noted, and seven patients remained on treatment for 1 year or longer.
  • [MeSH-major] Central Nervous System Neoplasms / drug therapy. Central Nervous System Neoplasms / mortality. Enzyme Inhibitors / pharmacokinetics. Farnesyltranstransferase / antagonists & inhibitors. Neoplasm Invasiveness / pathology. Piperidines / pharmacokinetics. Pyridines / pharmacokinetics
  • [MeSH-minor] Administration, Oral. Adolescent. Adult. Child. Child, Preschool. Dose-Response Relationship, Drug. Drug Administration Schedule. Female. Follow-Up Studies. Humans. Infant. Infant, Newborn. Male. Maximum Tolerated Dose. Neoplasm Staging. Risk Assessment. Survival Analysis. Treatment Outcome

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  • (PMID = 17634493.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / U01 CA81457
  • [Publication-type] Clinical Trial, Phase I; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Enzyme Inhibitors; 0 / Piperidines; 0 / Pyridines; 193275-84-2 / lonafarnib; EC 2.5.1.29 / Farnesyltranstransferase
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61. Hänninen MM, Haapasalo J, Haapasalo H, Fleming RE, Britton RS, Bacon BR, Parkkila S: Expression of iron-related genes in human brain and brain tumors. BMC Neurosci; 2009;10:36
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  • [Title] Expression of iron-related genes in human brain and brain tumors.
  • BACKGROUND: Defective iron homeostasis may be involved in the development of some diseases within the central nervous system.
  • Although the expression of genes involved in normal iron balance has been intensively studied in other tissues, little is known about their expression in the brain.
  • We investigated the mRNA levels of hepcidin (HAMP), HFE, neogenin (NEO1), transferrin receptor 1 (TFRC), transferrin receptor 2 (TFR2), and hemojuvelin (HFE2) in normal human brain, brain tumors, and astrocytoma cell lines.
  • The specimens included 5 normal brain tissue samples, 4 meningiomas, one medulloblastoma, 3 oligodendrocytic gliomas, 2 oligoastrocytic gliomas, 8 astrocytic gliomas, and 3 astrocytoma cell lines.
  • In most tumor types, the pattern of gene expression was diverse.
  • Notable findings include high expression of transferrin receptor 1 in the hippocampus and medulla oblongata compared to other brain regions, low expression of HFE in normal brain with elevated HFE expression in meningiomas, and absence of hepcidin mRNA in astrocytoma cell lines despite expression in normal brain and tumor specimens.
  • CONCLUSION: These results indicate that several iron-related genes are expressed in normal brain, and that their expression may be dysregulated in brain tumors.
  • [MeSH-major] Brain / metabolism. Brain Neoplasms / metabolism. Gene Expression Regulation, Neoplastic / physiology. Histocompatibility Antigens Class I / metabolism. Membrane Proteins / metabolism
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antigens, CD / genetics. Antigens, CD / metabolism. Antimicrobial Cationic Peptides / genetics. Antimicrobial Cationic Peptides / metabolism. Astrocytoma / genetics. Astrocytoma / metabolism. Cell Line, Tumor. Female. GPI-Linked Proteins. Hepcidins. Humans. Male. Meningioma / genetics. Meningioma / metabolism. Middle Aged. Oligodendroglioma / genetics. Oligodendroglioma / metabolism. RNA, Messenger / analysis. Receptors, Transferrin / genetics. Receptors, Transferrin / metabolism. Statistics, Nonparametric. Young Adult

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  • (PMID = 19386095.001).
  • [ISSN] 1471-2202
  • [Journal-full-title] BMC neuroscience
  • [ISO-abbreviation] BMC Neurosci
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antimicrobial Cationic Peptides; 0 / CD71 antigen; 0 / GPI-Linked Proteins; 0 / HAMP protein, human; 0 / HFE protein, human; 0 / HFE2 protein, human; 0 / Hepcidins; 0 / Histocompatibility Antigens Class I; 0 / Membrane Proteins; 0 / RNA, Messenger; 0 / Receptors, Transferrin; 0 / TFR2 protein, human; 0 / neogenin
  • [Other-IDs] NLM/ PMC2679039
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62. Korshunov A, Benner A, Remke M, Lichter P, von Deimling A, Pfister S: Accumulation of genomic aberrations during clinical progression of medulloblastoma. Acta Neuropathol; 2008 Oct;116(4):383-90
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  • [Title] Accumulation of genomic aberrations during clinical progression of medulloblastoma.
  • Medulloblastomas comprise the most frequent malignant brain tumor in childhood and one of the biggest challenges in pediatric oncology.
  • The current concept suggests that these tumors may undergo stepwise progression as it has been shown for other brain tumors.
  • However, conclusive evidence of molecular progression over time has not been demonstrated yet for medulloblastoma.
  • In the present study, 28 pairs of medulloblastoma at primary diagnosis and at the time of recurrence, either occurring as local tumor regrowth or tumor dissemination, were histopathologically and molecularly analyzed.
  • These results suggest that early recurrence in medulloblastoma mainly occurs in tumors with a highly malignant genotype and phenotype per se, whereas late recurrence is often dependent on tumor evolution toward a more malignant biology.
  • Therefore, biopsy of recurrent tumors should be performed to assess the biologic properties of the relapsed tumor, especially when targeted therapy approaches are considered.
  • [MeSH-major] Cerebellar Neoplasms / genetics. Chromosome Aberrations. Chromosomes, Human, Pair 17 / genetics. Chromosomes, Human, Pair 6 / genetics. Medulloblastoma / genetics. Nuclear Proteins / genetics. Oncogene Proteins / genetics. Proto-Oncogene Proteins c-myc / genetics
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / genetics. Biomarkers, Tumor / metabolism. Child. Child, Preschool. Cytogenetic Analysis. Disease Progression. Female. Humans. Male. Neoplasm Recurrence, Local / genetics. Prognosis

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  • (PMID = 18704466.001).
  • [ISSN] 1432-0533
  • [Journal-full-title] Acta neuropathologica
  • [ISO-abbreviation] Acta Neuropathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / MYC protein, human; 0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / Proto-Oncogene Proteins c-myc
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63. Valera ET, Lucio-Eterovic AK, Neder L, Scrideli CA, Machado HR, Carlotti-Junior CG, Queiroz RG, Motta FJ, Tone LG: Quantitative PCR analysis of the expression profile of genes related to multiple drug resistance in tumors of the central nervous system. J Neurooncol; 2007 Oct;85(1):1-10
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  • [Title] Quantitative PCR analysis of the expression profile of genes related to multiple drug resistance in tumors of the central nervous system.
  • OBJECTIVES: To evaluate and compare the profile of expression of genes related to drug resistance in brain tumors and to analyze the impact of the increased expression of these genes on overall survival.
  • METHODS: Eighty microdissected brain tumor samples from 79 patients were analyzed by RQ-PCR for the genes MDR1, MRP1, MRP3, LRP and BCRP.
  • Pediatric cases (0 to 20 years): 46 (17F:29M, median age 7.3 +/- 5.9 years); adult tumors: 33 (17F:16M, median age 46.6 +/- 14.5 years).
  • Histological diagnoses: 21 astrocytomas I and II, 28 astrocytomas III and glioblastomas, 17 medulloblastomas, 8 ependymomas, and 6 oligodendrogliomas.
  • The MRP1 gene was preferentially expressed by medulloblastomas (P = 0.04) and ependymomas (P = 0.04); ependymomas also presented an increase of LRP (P = 0.02).
  • Increased expression of resistance genes, separately or jointly, was not correlated with shorter overall survival in patients with medulloblastomas/PNET and malignant gliomas.
  • The increased expression of resistance genes had no impact on the overall survival of patients with medulloblastomas/PNET and high grade gliomas.
  • [MeSH-major] Central Nervous System Neoplasms / genetics. Drug Resistance, Multiple / genetics. Drug Resistance, Neoplasm / genetics. Gene Expression Regulation, Neoplastic / genetics. Gene Expression Regulation, Neoplastic / physiology. Reverse Transcriptase Polymerase Chain Reaction / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Brain Neoplasms / drug therapy. Brain Neoplasms / genetics. Brain Neoplasms / mortality. Calibration. Child. Child, Preschool. DNA Primers. Female. Gene Expression Profiling. Glioma / drug therapy. Glioma / genetics. Glioma / mortality. Humans. Immunohistochemistry. Infant. Male. Medulloblastoma / drug therapy. Medulloblastoma / genetics. Medulloblastoma / mortality. Middle Aged. RNA, Neoplasm / biosynthesis. RNA, Neoplasm / genetics. Survival Analysis

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  • (PMID = 17429576.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA Primers; 0 / RNA, Neoplasm
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64. Takei H, Bhattacharjee MB, Rivera A, Dancer Y, Powell SZ: New immunohistochemical markers in the evaluation of central nervous system tumors: a review of 7 selected adult and pediatric brain tumors. Arch Pathol Lab Med; 2007 Feb;131(2):234-41
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  • [Title] New immunohistochemical markers in the evaluation of central nervous system tumors: a review of 7 selected adult and pediatric brain tumors.
  • CONTEXT: Immunohistochemistry (IHC) has become an important tool in the diagnosis of brain tumors.
  • We discuss (1) placental alkaline phosphatase, c-Kit, and OCT4 for germinoma, (2) alpha-inhibin and D2-40 for capillary hemangioblastoma, (3) phosphohistone-H3 (PHH3), MIB-1/Ki-67, and claudin-1 for meningioma, (4) PHH3, MIB-1/Ki-67, and p53 for astrocytoma, (5) synaptophysin, microtubule-associated protein 2, neurofilament protein, and neuronal nuclei for medulloblastoma, (6) INI1 for atypical teratoid/rhabdoid tumor, and (7) epithelial membrane antigen for ependymoma.
  • All the markers presented here are used mainly for supporting or confirming the diagnosis, with the exception of the proliferation markers (MIB-1/Ki-67 and PHH3), which are primarily used to support grading and are reportedly associated with prognosis in certain categories of brain tumors.
  • In addition, IHC is also of great help in predicting the prognosis for certain brain tumors.
  • [MeSH-major] Biomarkers, Tumor / analysis. Central Nervous System Neoplasms / diagnosis. Central Nervous System Neoplasms / metabolism. Immunohistochemistry
  • [MeSH-minor] Adult. Antibodies. Astrocytoma / diagnosis. Astrocytoma / metabolism. Child. Diagnosis, Differential. Ependymoma / diagnosis. Ependymoma / metabolism. Germinoma / diagnosis. Germinoma / metabolism. Hemangioblastoma / diagnosis. Hemangioblastoma / metabolism. Humans. Medulloblastoma / diagnosis. Medulloblastoma / metabolism. Meningioma / diagnosis. Meningioma / metabolism. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / metabolism

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  • (PMID = 17284108.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies; 0 / Biomarkers, Tumor
  • [Number-of-references] 96
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65. Torii K, Tsuyuguchi N, Kawabe J, Sunada I, Hara M, Shiomi S: Correlation of amino-acid uptake using methionine PET and histological classifications in various gliomas. Ann Nucl Med; 2005 Dec;19(8):677-83
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  • OBJECTIVE: The uptake of L-methyl-11C-methionine (MET) by gliomas is greater than that by intact tissue, making methionine very useful for evaluation of tumor extent.
  • If the degree of malignancy of brain tumors can be evaluated by MET-PET, the usefulness of MET-PET as a means of diagnosing brain tumors will increase.
  • Tumors included diffuse astrocytoma, anaplastic astrocytoma, glioblastoma, ependymoma, oligodendroglioma, medulloblastoma, dysembryoplastic neuroepithelial tumor, choroid plexus papilloma, central neurocytoma, optic glioma, gliomatosis cerebri, pleomorphic xanthoastrocytoma, and ganglioglioma.
  • Tumor activity and degree of malignancy were evaluated using Ki-67LI (LI: labeling index) and Kaplan-Meier survival curves.
  • The correlations between methionine uptake and tumor proliferation (tumor versus contralateral gray matter ratio (T/N) and Ki-67LI) were determined for the group of all subjects.
  • Ki-67LI differed significantly between the high-grade group and low-grade group at T/N levels between 1.5 and 1.8 on analysis using tumor proliferative potential (p = 0.019-0.031).
  • CONCLUSIONS: When analysis was confined to cases of astrocytic tumor, a correlation was noted between methionine accumulation and Ki-67LI.
  • [MeSH-major] Brain Neoplasms / pathology. Brain Neoplasms / radionuclide imaging. Glioma / pathology. Glioma / radionuclide imaging. Methionine / pharmacokinetics. Positron-Emission Tomography / methods
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Female. Humans. Image Interpretation, Computer-Assisted / methods. Male. Middle Aged. ROC Curve. Radiopharmaceuticals / pharmacokinetics. Reproducibility of Results. Sensitivity and Specificity. Statistics as Topic. Tissue Distribution


66. Olson MV, Johnson DG, Jiang H, Xu J, Alonso MM, Aldape KD, Fuller GN, Bekele BN, Yung WK, Gomez-Manzano C, Fueyo J: Transgenic E2F1 expression in the mouse brain induces a human-like bimodal pattern of tumors. Cancer Res; 2007 May 1;67(9):4005-9
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  • [Title] Transgenic E2F1 expression in the mouse brain induces a human-like bimodal pattern of tumors.
  • The Rb/E2F pathway is deregulated in most human brain tumors, and the finding that loss of E2F1 reduced pituitary tumorigenesis in Rb(+/-) mice suggests that loss of pRb induces brain tumors by activating E2F1.
  • We therefore investigated the role of E2F1 in the development and maintenance of brain cancer using a transgenic mouse model engineered to express E2F1 specifically within glial cells (GFAP-tgE2F1).
  • GFAP-tgE2F1 mice developed a highly penetrant phenotype characterized by neurologic defects, and examination of the brains revealed the presence of brain tumors in 20% of these animals.
  • Importantly, the distribution of tumors according to mouse age suggests the existence of a bimodal pattern of tumor development, forcing a comparison with the human disease.
  • Mice, at an early age, with deregulated E2F1 show the formation of embryonal brain tumors such as medulloblastoma, choroid plexus carcinoma, and primary neuroectodermal tumor.
  • Conversely, at an older age, mice escaping embryonal tumor formation present with malignant gliomas, which are typically identified in the human adult population.
  • Thus, this study offers the first evidence for a global role of E2F1 in the formation and maintenance of multilineage brain tumors, irrefutably establishing E2F1 as an oncogene in the brain.
  • [MeSH-major] Brain Neoplasms / genetics. Cell Transformation, Neoplastic / genetics. E2F1 Transcription Factor / genetics

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  • (PMID = 17483310.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA16672; United States / NIEHS NIH HHS / ES / ES007784; United States / NCI NIH HHS / CA / R01CA79648
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / E2F1 Transcription Factor; 0 / E2F1 protein, human
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67. Yasuda K, Taguchi H, Sawamura Y, Ikeda J, Aoyama H, Fujieda K, Ishii N, Kashiwamura M, Iwasaki Y, Shirato H: Low-dose craniospinal irradiation and ifosfamide, cisplatin and etoposide for non-metastatic embryonal tumors in the central nervous system. Jpn J Clin Oncol; 2008 Jul;38(7):486-92
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  • [Title] Low-dose craniospinal irradiation and ifosfamide, cisplatin and etoposide for non-metastatic embryonal tumors in the central nervous system.
  • OBJECTIVE: The current study was conducted to evaluate the effects of low-dose craniospinal irradiation (CSI) combined with chemotherapy on non-metastatic embryonal tumors in the central nervous system (CNS), including medulloblastoma and supra-tentorial primitive neuroectodermal tumors (ST-PNET).
  • The dose to the primary tumor bed was 39.6-54 Gy.
  • Both 5-year OAS and PFS were 82% (CI: 59-100%) for the patients with medulloblastoma and 80% (CI: 45-100%) for the patients with ST-PNET.
  • CONCLUSION: Low-dose CSI and ICE chemotherapy may have a role as a treatment option for a subset of patients with non-metastatic embryonal tumors in the CNS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Brain Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy. Neuroectodermal Tumors, Primitive / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Dose-Response Relationship, Radiation. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Infant. Male. Radiotherapy Dosage. Supratentorial Neoplasms / pathology. Supratentorial Neoplasms / therapy. Survival Analysis

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  • (PMID = 18573848.001).
  • [ISSN] 1465-3621
  • [Journal-full-title] Japanese journal of clinical oncology
  • [ISO-abbreviation] Jpn. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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68. Privitera G, Acquaviva G, Ettorre GC, Spatola C: Antiangiogenic therapy in the treatment of recurrent medulloblastoma in the adult: case report and review of the literature. J Oncol; 2009;2009:247873
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  • [Title] Antiangiogenic therapy in the treatment of recurrent medulloblastoma in the adult: case report and review of the literature.
  • Medulloblastoma is a rare tumor in central nervous system, with an even rarer occurrence in adulthood.
  • We report the case of a 51-year-old man with recurrent medulloblastoma.
  • The aim of this report is to show that recurrent medulloblastoma in adults can be approached with a multimodality treatment and that antiangiogenic therapy should have a role in the management of this disease.

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  • [Cites] J Neurol. 2005 Mar;252(3):291-9 [16189725.001]
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  • (PMID = 20111585.001).
  • [ISSN] 1687-8469
  • [Journal-full-title] Journal of oncology
  • [ISO-abbreviation] J Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Other-IDs] NLM/ PMC2804042
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69. Eskandary H, Sabba M, Khajehpour F, Eskandari M: Incidental findings in brain computed tomography scans of 3000 head trauma patients. Surg Neurol; 2005 Jun;63(6):550-3; discussion 553
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  • [Title] Incidental findings in brain computed tomography scans of 3000 head trauma patients.
  • We studied the frequency of incidental findings on 3000 brain CT scans of trauma patients.
  • METHODS: Three thousands standard brain CT scans of trauma patients were evaluated for some incidental findings.
  • RESULTS: In this study we found 30 incidental abnormalities that include 8 cases of tumor: 3 meningioma, 2 craniopharyngioma, 1 oligodendroglioma, 1 low-grade astrocytoma, and 1 medulloblastoma.
  • Three suspect lipomas were found in midline and near midline of the brain.
  • CONCLUSION: Cisterna magna enlargement was the most common incidental finding and brain tumor and arachnoid cyst were next in frequency.
  • [MeSH-major] Brain / radiography. Brain Diseases / radiography. Brain Neoplasms / radiography. Craniocerebral Trauma / radiography. Tomography, X-Ray Computed / statistics & numerical data
  • [MeSH-minor] Adolescent. Adult. Aged. Arachnoid Cysts / pathology. Arachnoid Cysts / radiography. Calcinosis / pathology. Calcinosis / radiography. Child. Child, Preschool. Comorbidity. Cross-Sectional Studies. Female. Humans. Hydrocephalus / pathology. Hydrocephalus / radiography. Infant. Infant, Newborn. Male. Middle Aged. Prevalence


70. Furtado SV, Venkatesh PK, Dadlani R, Reddy K, Hegde AS: Adult medulloblastoma and the "dural-tail" sign: rare mimic of a posterior petrous meningioma. Clin Neurol Neurosurg; 2009 Jul;111(6):540-3
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  • [Title] Adult medulloblastoma and the "dural-tail" sign: rare mimic of a posterior petrous meningioma.
  • The histological diagnosis was classical medulloblastoma.
  • We review literature of this atypical presentation of medulloblastoma and "dural-tail" sign, which can be associated with other benign or malignant lesions.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Cerebellopontine Angle / pathology. Dura Mater / pathology. Infratentorial Neoplasms / pathology. Medulloblastoma / pathology. Meningioma / pathology
  • [MeSH-minor] Adult. Humans. Magnetic Resonance Spectroscopy. Male. Petrous Bone. Treatment Outcome

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  • (PMID = 19285790.001).
  • [ISSN] 1872-6968
  • [Journal-full-title] Clinical neurology and neurosurgery
  • [ISO-abbreviation] Clin Neurol Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Netherlands
  • [Number-of-references] 19
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71. Nakano I, Masterman-Smith M, Saigusa K, Paucar AA, Horvath S, Shoemaker L, Watanabe M, Negro A, Bajpai R, Howes A, Lelievre V, Waschek JA, Lazareff JA, Freije WA, Liau LM, Gilbertson RJ, Cloughesy TF, Geschwind DH, Nelson SF, Mischel PS, Terskikh AV, Kornblum HI: Maternal embryonic leucine zipper kinase is a key regulator of the proliferation of malignant brain tumors, including brain tumor stem cells. J Neurosci Res; 2008 Jan;86(1):48-60
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  • [Title] Maternal embryonic leucine zipper kinase is a key regulator of the proliferation of malignant brain tumors, including brain tumor stem cells.
  • Emerging evidence suggests that neural stem cells and brain tumors regulate their proliferation via similar pathways.
  • Here we describe how MELK expression is correlated with pathologic grade of brain tumors, and its expression levels are significantly correlated with shorter survival, particularly in younger glioblastoma patients.
  • In primary cultures from human glioblastoma and medulloblastoma, MELK knockdown by siRNA results in inhibition of the proliferation and survival of these tumors.
  • These results demonstrate a critical role for MELK in the proliferation of brain tumors, including their stem cells, and suggest that MELK may be a compelling molecular target for treatment of high-grade brain tumors.
  • [MeSH-major] Brain Neoplasms / pathology. Cell Proliferation. Glioblastoma / pathology. Neoplastic Stem Cells / physiology. Protein-Serine-Threonine Kinases / physiology
  • [MeSH-minor] Adult. Aged. Animals. Cells, Cultured. Female. Flow Cytometry / methods. Gene Expression Regulation, Neoplastic / drug effects. Humans. Male. Mass Spectrometry / methods. Mice. Mice, Knockout. Middle Aged. Pituitary Adenylate Cyclase-Activating Polypeptide / deficiency. RNA, Small Interfering / pharmacology. Receptors, Cell Surface / deficiency. Transfection / methods

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  • (PMID = 17722061.001).
  • [ISSN] 0360-4012
  • [Journal-full-title] Journal of neuroscience research
  • [ISO-abbreviation] J. Neurosci. Res.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA108633; United States / NCI NIH HHS / CA / CA110384; United States / NCI NIH HHS / CA / CA88173; United States / NICHD NIH HHS / HD / HD34475; United States / NINDS NIH HHS / NS / NS050151; United States / NINDS NIH HHS / NS / NS43147
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adcyap1 protein, mouse; 0 / Pituitary Adenylate Cyclase-Activating Polypeptide; 0 / RNA, Small Interfering; 0 / Receptors, Cell Surface; 0 / patched receptors; EC 2.7.1.- / MELK protein, human; EC 2.7.11.1 / Protein-Serine-Threonine Kinases
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72. Rodriguez FJ, Eberhart C, O'Neill BP, Slezak J, Burger PC, Goldthwaite P, Wu W, Giannini C: Histopathologic grading of adult medulloblastomas. Cancer; 2007 Jun 15;109(12):2557-65
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  • [Title] Histopathologic grading of adult medulloblastomas.
  • BACKGROUND: Histopathologic evaluation of the degree and extent of anaplasia is a useful prognostic parameter in pediatric medulloblastomas.
  • Whether the same applies to adult medulloblastomas is not known.
  • METHODS: The study included 74 adult patients with histologically confirmed medulloblastomas and retrospectively reassessed 67 cases with available slides for the presence of nodularity, collagen deposition (desmoplasia without nodules), and degree and extent of anaplasia.
  • CONCLUSIONS: The incidence of severe anaplasia in adult medulloblastomas is lower than in the pediatric population.
  • [MeSH-major] Cerebellar Neoplasms / classification. Medulloblastoma / classification
  • [MeSH-minor] Adolescent. Adult. Age Distribution. Aged. Female. Humans. Immunoenzyme Techniques. Incidence. Male. Middle Aged. Neoplasm Staging. Prognosis. Retrospective Studies. Sex Distribution. Survival Rate

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  • [Copyright] Copyright 2007 American Cancer Society.
  • (PMID = 17487854.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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73. Srivastava NK, Pradhan S, Gowda GA, Kumar R: In vitro, high-resolution 1H and 31P NMR based analysis of the lipid components in the tissue, serum, and CSF of the patients with primary brain tumors: one possible diagnostic view. NMR Biomed; 2010 Feb;23(2):113-22
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  • [Title] In vitro, high-resolution 1H and 31P NMR based analysis of the lipid components in the tissue, serum, and CSF of the patients with primary brain tumors: one possible diagnostic view.
  • In vitro, high-resolution (1)H and (31)P NMR based qualitative and quantitative analyses of the lipid components of the tissue, serum, and CSF of patients with primary brain tumors were performed.
  • Proton NMR spectra of the lipid extract of serum (blood specimen collected before the surgical procedure) and surgically discarded tissue showed that the total cholesterol (T.CHOL) and choline containing phospholipids (PL) were significantly higher in quantity in medulloblastoma and glioblastoma multiforme as compared to normal subjects.
  • There was a reduction in the quantity of CHOLest in the serum lipid extract of the tumor patients as compared to normal subjects.
  • Ratio of PL to T.CHOL in serum lipid extract showed a significant difference between different grades of tumors versus normal subjects, while, a significant difference was observed only in medulloblastoma versus normal subjects in tissue lipid extract.
  • Ratio of CHOL to CHOLest distinguishes the different grades of tumors versus normal subjects as well as between different grades of tumors (except medulloblastoma versus glioblastoma).
  • The ratio of the Ph (total phospholipids except phosphatidylcholine) to PC (phosphatidylcholine) in (31)P NMR based study showed a significant difference in all grades of tumors (except medulloblastoma) in normal subjects in tissue lipid extract as well as between different grades of tumors.
  • Medulloblastoma could be differentiated from glioblastoma as well as from normal subjects in serum lipid extract by the ratio of the Ph to PC.
  • PL and T.CHOL provided discrimination between different grades of tumors (except glioblastoma versus medulloblastoma) in the lipid extract of the CSF.
  • This study suggests the role of lipid estimation in CSF and serum as a complementary diagnostic tool for the evaluation of brain tumors preoperatively.
  • NMR-based lipid estimation of post-surgical tumor tissue may also contribute to differentiating the tumor types.
  • [MeSH-major] Brain Neoplasms / blood. Brain Neoplasms / cerebrospinal fluid. Lipids / analysis. Magnetic Resonance Spectroscopy / methods
  • [MeSH-minor] Adult. Cholesterol / analysis. Cholesterol / blood. Cholesterol / cerebrospinal fluid. Female. Humans. Male. Phospholipids / analysis. Phospholipids / blood. Phospholipids / cerebrospinal fluid. Tissue Extracts

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  • [Copyright] (c) 2009 John Wiley & Sons, Ltd.
  • (PMID = 19774696.001).
  • [ISSN] 1099-1492
  • [Journal-full-title] NMR in biomedicine
  • [ISO-abbreviation] NMR Biomed
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Lipids; 0 / Phospholipids; 0 / Tissue Extracts; 97C5T2UQ7J / Cholesterol
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74. Khalil EM: Treatment results of adults and children with medulloblastoma NCI, Cairo University experience. J Egypt Natl Canc Inst; 2008 Jun;20(2):175-86
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  • [Title] Treatment results of adults and children with medulloblastoma NCI, Cairo University experience.
  • PURPOSE: To evaluate treatment outcome and prognostic factors of adults and pediatric medulloblastoma patients treated by adjuvant postoperative craniospinal irradiation (CSI) and chemotherapy.
  • PATIENTS AND METHODS: Between 1997 and 2004, 67 patients were treated in the National cancer Institute- Cairo University; 51 pediatric patients with a median age of 7 years and 16 adult patients with a median age of 25 years.
  • According to the Chang staging system; 50%-35% , 37.5%-47% and 12.5%-18% had T2, T3 and T4 tumors of adults and pediatric patient's population respectively.
  • All patients underwent primary surgical resection; near total resection in 25% , Subtotal resection in 61% ; with tumor residual < 1.5cm(2) in 49% compared to 51% with > 1.5cm(2) residual tumor and 14% , had biopsy only.
  • All patients were treated by craniospinal radiotherapy (RT); with a median dose of 34Gy to the whole brain, 54Gy to the posterior fossa and 32Gy to the spinal axis.
  • The median interval between surgery and RT was 45 days and 38 days for the pediatric and adult groups respectively.
  • The median duration of RT was 54 days and 52 days for pediatric and adult patients respectively.
  • RESULTS: For the pediatric and adult patients, the 5- and 7-year overall and disease-free survival rates were 89% & 78% vs. 84% & 56% and 80% & 68% vs. 79% & 52% respectively.
  • Fourteen patients (21% ) relapsed (10 pediatric and 4 adults) at a median time of 11 months vs. 23 months and a median follow-up period of 8 and 12 months respectively; Neuro-axis was the most common site of relapse (11 patients).
  • Ninety percent (9/10) of the pediatric relapses were of the high risk group (8 received no chemotherapy) and took place within 2 years; similarly all adult relapses were of the high risk group; three relapses took place after 2 years.
  • For adult patients; only the risk category was a significant prognostic factor with 5-year disease-free survival rate of 100% vs. 40% for low and high risk respectively (p=0.03).
  • CONCLUSION: Survival rates of medulloblastoma pediatric patients were better than the adult ones.
  • Late relapses, lateral tumor location and shorter median follow up were noted in adult patients.
  • Advanced tumor stage, metastases at presentation, limited tumor resection were powerful prognostic factors among the pediatric patients.
  • In addition, high risk category was shown to be a prognostic factor for both pediatric and adult patients.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Cerebellar Neoplasms / therapy. Cranial Irradiation. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / therapy. Neoplasm Staging. Prognosis. Radiotherapy Dosage. Radiotherapy, Adjuvant. Survival Rate. Treatment Outcome. Young Adult

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  • (PMID = 20029474.001).
  • [ISSN] 1110-0362
  • [Journal-full-title] Journal of the Egyptian National Cancer Institute
  • [ISO-abbreviation] J Egypt Natl Canc Inst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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75. Herrlinger U, Steinbrecher A, Rieger J, Hau P, Kortmann RD, Meyermann R, Schabet M, Bamberg M, Dichgans J, Bogdahn U, Weller M: Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse. J Neurol; 2005 Mar;252(3):291-9
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  • [Title] Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.
  • Adult medulloblastoma is a rare tumor with few retrospective studies published so far.
  • This study reports therapy and outcome in all adult (>or=16 years old) medulloblastoma (n=34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n=2) treated in 2 neuro-oncological centers between 1976 and 2002.
  • In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients.
  • As in pediatric medulloblastoma patients, primary infiltration of the floor of the 4(th) ventricle indicates a poor prognosis.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Analysis of Variance. Combined Modality Therapy. Demography. Disease Progression. Disease-Free Survival. Dose-Response Relationship, Radiation. Drug Therapy / methods. Female. Humans. Male. Middle Aged. Radiotherapy, High-Energy / methods. Recurrence. Regression Analysis. Retrospective Studies. Risk Factors. Time Factors. Treatment Outcome

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  • (PMID = 16189725.001).
  • [ISSN] 0340-5354
  • [Journal-full-title] Journal of neurology
  • [ISO-abbreviation] J. Neurol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Germany
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76. Ferretti E, De Smaele E, Po A, Di Marcotullio L, Tosi E, Espinola MS, Di Rocco C, Riccardi R, Giangaspero F, Farcomeni A, Nofroni I, Laneve P, Gioia U, Caffarelli E, Bozzoni I, Screpanti I, Gulino A: MicroRNA profiling in human medulloblastoma. Int J Cancer; 2009 Feb 01;124(3):568-77
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  • [Title] MicroRNA profiling in human medulloblastoma.
  • Medulloblastoma is an aggressive brain malignancy with high incidence in childhood.
  • However, no data are yet available on human primary medulloblastomas.
  • A high throughput microRNA expression profiles was performed in human primary medulloblastoma specimens to investigate microRNA involvement in medulloblastoma carcinogenesis.
  • We identified specific microRNA expression patterns which distinguish medulloblastoma differing in histotypes (anaplastic, classic and desmoplastic), in molecular features (ErbB2 or c-Myc overexpressing tumors) and in disease-risk stratification.
  • MicroRNAs expression profile clearly differentiates medulloblastoma from either adult or fetal normal cerebellar tissues.
  • Only a few microRNAs displayed upregulated expression, while most of them were downregulated in tumor samples, suggesting a tumor growth-inhibitory function.
  • This property has been addressed for miR-9 and miR-125a, whose rescued expression promoted medulloblastoma cell growth arrest and apoptosis while targeting the proproliferative truncated TrkC isoform.
  • In conclusion, misregulated microRNA expression profiles characterize human medulloblastomas, and may provide potential targets for novel therapeutic strategies.
  • [MeSH-major] Biomarkers, Tumor / genetics. Cerebellar Neoplasms / genetics. Gene Expression Profiling. Medulloblastoma / genetics. MicroRNAs

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  • [Copyright] Copyright (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18973228.001).
  • [ISSN] 1097-0215
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Grant] Italy / Telethon / / GGP07118
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / MicroRNAs; EC 2.7.10.1 / Receptor, trkC
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77. Eberhart CG, Chaudhry A, Daniel RW, Khaki L, Shah KV, Gravitt PE: Increased p53 immunopositivity in anaplastic medulloblastoma and supratentorial PNET is not caused by JC virus. BMC Cancer; 2005 Feb 17;5:19
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  • [Title] Increased p53 immunopositivity in anaplastic medulloblastoma and supratentorial PNET is not caused by JC virus.
  • BACKGROUND: p53 mutations are relatively uncommon in medulloblastoma, but abnormalities in this cell cycle pathway have been associated with anaplasia and worse clinical outcomes.
  • We correlated p53 protein expression with pathological subtype and clinical outcome in 75 embryonal brain tumors.
  • METHODS: p53 protein levels were evaluated semi-quantitatively in 64 medulloblastomas, 3 atypical teratoid rhabdoid tumors (ATRT), and 8 supratentorial primitive neuroectodermal tumors (sPNET) using immunohistochemistry.
  • JC viral sequences were analyzed in DNA extracted from 33 frozen medulloblastoma and PNET samples using quantitative polymerase chain reaction.
  • RESULTS: p53 expression was detected in 18% of non-anaplastic medulloblastomas, 45% of anaplastic medulloblastomas, 67% of ATRT, and 88% of sPNET.
  • The increased p53 immunoreactivity in anaplastic medulloblastoma, ATRT, and sPNET was statistically significant.
  • No JC virus was identified in the embryonal brain tumor samples, while an endogenous human retrovirus (ERV-3) was readily detected.
  • CONCLUSION: Immunoreactivity for p53 protein is more common in anaplastic medulloblastomas, ATRT and sPNET than in non-anaplastic tumors, and is associated with worse clinical outcomes.

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  • (PMID = 15717928.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] ENG
  • [Grant] United States / NINDS NIH HHS / NS / K08 NS043279; United States / NINDS NIH HHS / NS / K08NS43279
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Tumor Suppressor Protein p53
  • [Other-IDs] NLM/ PMC554768
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78. Behdad A, Perry A: Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol; 2010 Mar;20(2):441-50
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  • [Title] Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases.
  • Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) include supratentorial, brain stem, and spinal cord tumors with medulloblastoma-like histopathology.
  • After re-diagnosis of three infantile cases as atypical teratoid/rhabdoid tumor (AT/RT), 33 remaining CNS PNETs were retrieved for clinicopathologic and fluorescence in situ hybridization studies.
  • We conclude that in CNS PNET: (i) routine application of INI1 immunohistochemistry helps rule out AT/RT, particularly in infants;.
  • (iii) involvement of CNS parenchyma by Ewing sarcoma/peripheral PNET is rare enough that EWS gene testing is not necessary unless significant dural involvement is present; and (iv) both anaplastic/large cell features and polysomies of 2 and 8 are associated with more aggressive clinical behavior.
  • [MeSH-major] Brain Neoplasms / genetics. Brain Neoplasms / pathology. Neuroectodermal Tumors, Primitive / genetics. Neuroectodermal Tumors, Primitive / pathology. Spinal Cord Neoplasms / genetics. Spinal Cord Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aneuploidy. Child. Child, Preschool. Chromosomes, Human, Pair 2. Chromosomes, Human, Pair 22. Chromosomes, Human, Pair 8. Female. Humans. Infant. Male. Middle Aged. Nuclear Proteins / genetics. Oncogene Proteins / genetics. RNA-Binding Protein EWS / genetics. Rhabdoid Tumor / diagnosis. Rhabdoid Tumor / genetics. Rhabdoid Tumor / pathology. Teratoma / diagnosis. Teratoma / genetics. Teratoma / pathology. Young Adult

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  • (PMID = 19725831.001).
  • [ISSN] 1750-3639
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / RNA-Binding Protein EWS
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79. Giordana MT, D'Agostino C, Pollo B, Silvani A, Ferracini R, Paiolo A, Ghiglione P, Chiò A: Anaplasia is rare and does not influence prognosis in adult medulloblastoma. J Neuropathol Exp Neurol; 2005 Oct;64(10):869-74
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  • [Title] Anaplasia is rare and does not influence prognosis in adult medulloblastoma.
  • Histopathologic grading based on increasing anaplasia predicts clinical behavior of pediatric medulloblastomas.
  • The present study was aimed at grading 86 medulloblastomas of adult patients (aged 18 and older) by anaplasia and analyzing the predictive power.
  • Severe nuclear pleomorphism was found in 4 of 86 cases; the only large-cell medulloblastoma was from an 18-year-old patient.
  • The histologic spectrum of medulloblastoma in adults is different from that in children.
  • [MeSH-major] Cerebellar Neoplasms / pathology. Medulloblastoma / pathology
  • [MeSH-minor] Adult. Aged. Anaplasia. Female. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Survival Analysis

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  • (PMID = 16215458.001).
  • [ISSN] 0022-3069
  • [Journal-full-title] Journal of neuropathology and experimental neurology
  • [ISO-abbreviation] J. Neuropathol. Exp. Neurol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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80. Riazmontazer N, Daneshbod Y: Cytology of desmoplastic medulloblastoma in imprint smears: a report of 2 cases. Acta Cytol; 2006 Jan-Feb;50(1):97-100
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  • [Title] Cytology of desmoplastic medulloblastoma in imprint smears: a report of 2 cases.
  • BACKGROUND: Desmoplastic medulloblastoma is a rare subtype of medulloblastoma with astroglial differentiation.
  • The cytomorphologic features in intraoperative imprint smears from 2 cases of desmoplastic medulloblastoma are described.
  • CASE REPORTS: A 22-year-old man and 27-year-old woman with a cerebellar tumor underwent craniotomy and tumor resection.
  • The cytology was misinterpreted as glial tumors, while the final histologic diagnosis in both cases were desmoplastic medulloblastoma.
  • CONCLUSION: Desmoplastic medulloblastoma shows distinctive cytology in intraoperative smears.
  • However, the occurrence of this rare type in adults and the presence of astroglial elements in imprint smears may cause a cytologic misinterpretation as gliomas.
  • [MeSH-major] Cerebellar Neoplasms / diagnosis. Medulloblastoma / diagnosis
  • [MeSH-minor] Adult. Astrocytes / pathology. Brain Neoplasms / diagnosis. Diagnostic Errors. Female. Glioma / diagnosis. Humans. Intraoperative Period. Male. Neurons / pathology

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  • (PMID = 16514849.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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81. Massimino M, Gandola L, Collini P, Seregni E, Marchianò A, Serra A, Pignoli E, Spreafico F, Pallotti F, Terenziani M, Biassoni V, Bombardieri E, Fossati-Bellani F: Thyroid-stimulating hormone suppression for protection against hypothyroidism due to craniospinal irradiation for childhood medulloblastoma/primitive neuroectodermal tumor. Int J Radiat Oncol Biol Phys; 2007 Oct 1;69(2):404-10
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  • [Title] Thyroid-stimulating hormone suppression for protection against hypothyroidism due to craniospinal irradiation for childhood medulloblastoma/primitive neuroectodermal tumor.
  • Hence, our study was launched in 1998 to evaluate the protective effect of TSH suppression during CSI for medulloblastoma/primitive neuroectodermal tumor.
  • PATIENTS AND METHODS: From Jan 1998 to Feb 2001, a total of 37 euthyroid children scheduled for CSI for medulloblastoma/primitive neuroectodermal tumor underwent thyroid ultrasound and free triiodothyronine (FT3), free thyroxine (FT4), and TSH evaluation at the beginning and end of CSI.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Cranial Irradiation / adverse effects. Hypothyroidism / prevention & control. Medulloblastoma / radiotherapy. Neuroectodermal Tumors, Primitive / radiotherapy. Thyrotropin / antagonists & inhibitors
  • [MeSH-minor] Adolescent. Adult. Biomarkers / blood. Child. Child, Preschool. Disease-Free Survival. Female. Humans. Infant. Male. Radiotherapy Dosage. Thyroxine / blood. Triiodothyronine / blood


82. Cakar M, Aksoy S, Kilickap S, Harputluoglu H, Erman M: Procarbazine, lomustine, vincristine combination may be effective in adult medulloblastoma patients with systemic metastases. J Neurooncol; 2005 Nov;75(2):233-4
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  • [Title] Procarbazine, lomustine, vincristine combination may be effective in adult medulloblastoma patients with systemic metastases.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / secondary. Lomustine / therapeutic use. Lymphatic Metastasis / pathology. Medulloblastoma / drug therapy. Procarbazine / therapeutic use. Vincristine / therapeutic use
  • [MeSH-minor] Adult. Antineoplastic Agents / therapeutic use. Antineoplastic Agents, Alkylating / therapeutic use. Antineoplastic Agents, Phytogenic / therapeutic use. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Time Factors. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 16132496.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 35S93Y190K / Procarbazine; 5J49Q6B70F / Vincristine; 7BRF0Z81KG / Lomustine
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83. Benesch M, Siegler N, Hoff Kv, Lassay L, Kropshofer G, Müller H, Sommer C, Rutkowski S, Fleischhack G, Urban C: Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study. Anticancer Drugs; 2009 Oct;20(9):794-9
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  • [Title] Safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with recurrent or refractory brain tumors: a multi-institutional retrospective study.
  • This retrospective study aimed to evaluate the safety and toxicity of intrathecal liposomal cytarabine (Depocyte) in children and adolescents with refractory or recurrent brain tumors.
  • Nineteen heavily pretreated patients (males, n = 14; females, n = 5; median age at diagnosis 8.5 years; range, 1.4-22 years) were given intrathecal liposomal cytarabine on a compassionate use basis for recurrent refractory medulloblastoma (n = 12), mixed germ cell tumor (n = 2), central nervous system primitive neuroectodermal tumors of the pons (n = 1), anaplastic ependymoma (n = 1), anaplastic oligodendroglioma (n = 1), atypical teratoid rhabdoid tumor (n = 1), or rhabdoid papillary meningioma (n = 1).
  • In conclusion, although intrathecal liposomal cytarabine was generally well tolerated, it should be used cautiously and only with dexamethasone prophylaxis in extensively pretreated patients with recurrent brain tumors.
  • [MeSH-major] Antimetabolites, Antineoplastic / adverse effects. Brain Neoplasms / drug therapy. Cytarabine / administration & dosage. Cytarabine / adverse effects
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Compassionate Use Trials. Delayed-Action Preparations. Drug Resistance, Neoplasm. Female. Humans. Infant. Injections, Spinal. Liposomes / administration & dosage. Male. Retrospective Studies. Salvage Therapy. Young Adult

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  • (PMID = 19617818.001).
  • [ISSN] 1473-5741
  • [Journal-full-title] Anti-cancer drugs
  • [ISO-abbreviation] Anticancer Drugs
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Delayed-Action Preparations; 0 / Liposomes; 04079A1RDZ / Cytarabine
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84. Rollison DE, Utaipat U, Ryschkewitsch C, Hou J, Goldthwaite P, Daniel R, Helzlsouer KJ, Burger PC, Shah KV, Major EO: Investigation of human brain tumors for the presence of polyomavirus genome sequences by two independent laboratories. Int J Cancer; 2005 Feb 20;113(5):769-74
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  • [Title] Investigation of human brain tumors for the presence of polyomavirus genome sequences by two independent laboratories.
  • JC virus (JCV), BK virus (BKV) and simian virus 40 (SV40) may be associated with human brain tumors.
  • These polyomaviruses have been shown to induce brain tumors in experimentally infected animals.
  • Several studies have found polyomavirus genomic sequences in human brain tumor tissues by using polymerase chain reaction (PCR), while others have not.
  • To assess the role of polyomaviruses in human brain tumors and address inconsistencies of previous reports, we investigated the prevalence of viral sequences in a series of 225 brain tumor tissue specimens in 2 independent laboratories.
  • The JHU laboratory amplified DNA from 165/225 (73%) tumors, of which 1 tumor tested positive (for SV40).
  • Nucleotide sequences for JCV, BKV and SV40 are rarely present in a large series of adult and pediatric brain tumors.
  • [MeSH-major] Brain Neoplasms / virology. Polyomavirus Infections / virology. Tumor Virus Infections / virology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. BK Virus / genetics. BK Virus / isolation & purification. Blotting, Southern. Child. Child, Preschool. DNA, Neoplasm / genetics. Female. Genome, Viral. Glioma / diagnosis. Glioma / genetics. Glioma / virology. Humans. Infant. JC Virus / genetics. JC Virus / isolation & purification. Male. Medulloblastoma / diagnosis. Medulloblastoma / genetics. Medulloblastoma / virology. Meningioma / diagnosis. Meningioma / genetics. Meningioma / virology. Middle Aged. Polymerase Chain Reaction. RNA, Messenger / genetics. RNA, Viral / genetics. Reverse Transcriptase Polymerase Chain Reaction. Simian virus 40 / genetics. Simian virus 40 / isolation & purification

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  • [Copyright] (c) 2004 Wiley-Liss, Inc.
  • (PMID = 15499616.001).
  • [ISSN] 0020-7136
  • [Journal-full-title] International journal of cancer
  • [ISO-abbreviation] Int. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / RNA, Messenger; 0 / RNA, Viral
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85. Hargrave DR, Hargrave UA, Bouffet E: Quality of health information on the Internet in pediatric neuro-oncology. Neuro Oncol; 2006 Apr;8(2):175-82
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The Internet is now the single largest source of health information and is used by many patients and their families who are affected by childhood brain tumors.
  • To assess the quality of pediatric neuro-oncology information on the Internet, we used search engines to look for information on five common tumor types (brain stem glioma, craniopharyngioma, ependymoma, low-grade glioma, and medulloblastoma).
  • Few sites offered information in languages other than English, and readability statistics showed an average required reading level of U.S. grade 12+ (the suggested level being grades 6-8 for an adult audience).
  • [MeSH-major] Brain Neoplasms. Internet / standards. Medical Informatics / standards. Medical Oncology. Neurology


86. Spiller SE, Ravanpay AC, Hahn AW, Olson JM: Suberoylanilide hydroxamic acid is effective in preclinical studies of medulloblastoma. J Neurooncol; 2006 Sep;79(3):259-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Suberoylanilide hydroxamic acid is effective in preclinical studies of medulloblastoma.
  • PURPOSE: Suberoylanilide hydroxamic acid (SAHA) has been studied in adult solid and hematologic malignancies.
  • However, little information has been reported on the effects of SAHA on central nervous system (CNS) tumors including medulloblastoma, the most common malignant brain tumor in children.
  • We investigated SAHA in preclinical medulloblastoma models to determine its anti-cancer efficacy as well as its ability to affect intracranial lesions when administered systemically.
  • EXPERIMENTAL DESIGN AND RESULTS: Tissue culture studies were performed treating primary human fibroblasts, established medulloblastoma cell lines, and primary human medulloblastoma tumors with SAHA.
  • At 10 microM concentration, SAHA had little effect on normal fibroblasts but caused >90% apoptosis in cultured medulloblastoma cells.
  • Primary medulloblastomas from patients were sensitive to SAHA compared to vehicle alone in ex vivo studies.
  • In athymic mice with medulloblastoma xenograft tumors, oral SAHA resulted in apoptosis of tumor tissue and significantly slowed tumor growth.
  • In the ND2:Smo transgenic mouse medulloblastoma model, SAHA treatment caused significant apoptosis in these cerebellar tumors.
  • CONCLUSIONS: SAHA effectively induces cell death in established medulloblastoma cell lines, human patient primary tumor cultures, medulloblastoma xenografts and intracranial spontaneous medulloblastomas.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Cerebellar Neoplasms / drug therapy. Hydroxamic Acids / pharmacology. Medulloblastoma / drug therapy

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  • (PMID = 16645722.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA112350-01
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Hydroxamic Acids; 58IFB293JI / vorinostat
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87. Souweidane MM, Morgenstern PF, Christos PJ, Edgar MA, Khakoo Y, Rutka JT, Dunkel IJ: Intraoperative arachnoid and cerebrospinal fluid sampling in children with posterior fossa brain tumors. Neurosurgery; 2009 Jul;65(1):72-8; discussion 78
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  • [Title] Intraoperative arachnoid and cerebrospinal fluid sampling in children with posterior fossa brain tumors.
  • OBJECTIVE: This study was conducted to determine whether arachnoid tissue or cerebrospinal fluid (CSF) sampling is valuable for risk stratification in children with posterior fossa brain tumors.
  • METHODS: Arachnoid tissue and CSF from the cisterna magna (CSFCM) was sampled at the time of primary tumor resection.
  • Arachnoid infiltration and CSF cytology were found in 20.0% and 44.8%, respectively, for medulloblastoma/pineoblastoma (primitive neuroectodermal tumor), 6.9% and 3.6% for pilocytic astrocytoma, and 0.0% and 33.3% for ependymoma.
  • The 3-year EFS for patients with primitive neuroectodermal tumor who had positive arachnoid sampling was 33.3%, compared with 67.3% in patients who had no evidence of arachnoid infiltration (P = 0.26).
  • The 3-year EFS for patients with primitive neuroectodermal tumor who had positive CSFCM was 50.0% compared with 67.5% in patients who had negative cytological analysis of CSFCM (P = 0.07).
  • CONCLUSION: Intraoperative evidence of arachnoid infiltration or CSFCM dissemination in patients with posterior fossa brain tumors occurs at a variable frequency that is dependent on tumor type, correlates with conventional M stage, and may be predictive of outcome.
  • [MeSH-minor] Adolescent. Adult. Aged. Child. Child, Preschool. Cohort Studies. Female. Humans. Infant. Magnetic Resonance Imaging. Male. Middle Aged. Retrospective Studies. Young Adult

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  • (PMID = 19574827.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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88. Mondin V, Ferlito A, Devaney KO, Woolgar JA, Rinaldo A: A survey of metastatic central nervous system tumors to cervical lymph nodes. Eur Arch Otorhinolaryngol; 2010 Nov;267(11):1657-66
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  • [Title] A survey of metastatic central nervous system tumors to cervical lymph nodes.
  • In children, neck masses often prove to be developmental cysts; in adults, the recent onset of a neck mass can signal a metastasis from a head and neck squamous carcinoma.
  • Less often, both adults and children may present with cervical masses caused by either non-Hodgkin's lymphoma or Hodgkin's disease.
  • There are, of course, less frequently encountered differential diagnostic possibilities; one of the most uncommon of all is the possibility of metastasis from an intracranial tumor.
  • The present review examines the published experience with 128 tumors that gave rise to cervical node metastases in both adult and in pediatric patients.
  • While it is presumed that the blood-brain barrier blocks the spread of most tumors beyond the intracranial locale, this is speculative.
  • Although many of the cervical node metastases reported here arose after craniotomy (and, presumably, after breaching of the blood-brain barrier), some arose in the absence of any preceding surgical procedure.
  • Cervical node metastases may arise from glial tumors (including glioblastoma multiforme, in both adult and pediatric patients) and non-glial tumors (such as medulloblastoma in pediatric patients).
  • The history of a previous intracranial lesion is often the key to correct diagnosis, since, without prompting, neither the pathologist nor the radiologist is likely to think of a cervical node metastasis from a brain tumor when assessing a cervical mass of unknown etiology.
  • [MeSH-major] Central Nervous System Neoplasms / pathology. Head and Neck Neoplasms / secondary. Lymphatic Metastasis / pathology
  • [MeSH-minor] Blood-Brain Barrier. Humans

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